Download HO/1 (P) CLINICAL AND LABORATORY PROFILE OF PRIMARY

HO/1 (P) CLINICAL AND LABORATORY PROFILE OF
IMMUNODEFECIENCY DISORDERS
Rajesh Joshi, Manisha Patil, Mukesh Desai
C/2, Om Parshwanath Apts, Saibaba Nagar, Borivali (West), Mumbai-400092
Email: [email protected]
PRIMARY
Objective: Clinical and laboratory profile of primary immunodefeciency disorders. (PID)
Methods: Cohort study over 3 years 2009-2011. Children satisfying inclusion criteria were
evaluated. A thorough physical examination with specific consideration to presence of
tonsils,thrush,BCG iosis,organomegaly was done.Investigations done were hemogram to
define neutropenia,lymphopenia and thrombocytopenia and specific immunological
investigations when necessary like NBT,CD subset,immunoglobulin levels,CD 154,CD
18,granule release assay .Microbiological and radiological investigations were also done.
Results: Most common defect was NK cell defect [HLH(n=22); primary (n=8),
secondary(n=14)] followed by B cell defect [Hypogammaglobinaemia(n=5) ,common
variable immunodeficiency(n=3),IgA deficiency(n=3) , Hyper IgM syndrome(n=1) and IgG
subclass deficiency (n=1) ] ,combined B
and T cell defect [Severe combined
immunodeficiency(n=5),Autoimmune lymphoproliferative syndrome(n=1), HyperIgE
syndrome (n=2)]; phagocytic defect [Chronic granulomatous disease (n=4),Leucocyte
adhesion defect(n=1) ],T
cell
defect [Mendelian susceptibility to mycobacterial
diseases(MSMD,n=3),chronic mucocutaneous candididasis(n=1) ]
and
neutrophil
defect[congenital neutropenia, 1patient]. All defects were common in males. Common
presenting features were pneumonia[B cell(53.8%),combined B and T cell(50%),phagocytic
defect (60%)];skin abscesses [phagocytic(60%) and T cell defect(75%)] and CNS infections
[NK cell defect(45.45%)]. 3 patients each of HLH,CGD and ALPS had family members with
similar disorders. The other features were absent tonsils (n=2 SCID,n=1 each of CGD and
Hypogammaglobinaemia), BCGiosis (n= 2 of CGD,n=1 each of MSMD and B cell defect)
and malnutrition (n=30).Laborotory profile revealed lymphopenia (n=3,SCID), congenital
neutropenia (n=1), thrombocytopenia (n=14,HLH).46.15% patients with B cell defect had
low levels of CD19 and 62.5% with combined B and T cell defect had low levels of CD3.
Conclusion: Of 85 patients fulfilling clinical criteria of PID diagnosis was confirmed in 53
patients (62.4%). Certain findings give a clue to type of defect like pneumonia (B cell),absent
tonsils(SCID), bi/pancytopenia and presence of haemophagocytes(HLH) .
HO/2 (P) EFFECTS OF HYDROXYUREATHERAPY IN SICKLE CELL DISEASE
AND DIFFERENT TYPES OF DIFFERENT TYPES OF CRISIS
N.L Phuljhele, Arpita Roy Ghatak
Kanchanganga phase II, Danganiya, PO-RSU, Raipur, C.G.
Email: [email protected]
Introduction:Hdroxyurea (HU) is known to stimulate foetal haemoglobin (HbF) with the
increase in the red cell volume. Various studies have shown beneficial effect of HU in sickle
cell disease (SCD) in adults. Aim: The aim of the study was to evaluate efficacy and toxicity
of HU therapy in children. Method:Patients with frequent vascular occlusion crisis (VOCs) or
with severe anaemia (Hb<7gm%), or stroke or transient ischemic attack were included in the
study. Other inclusion criteria were creatinine level <1.7gm%, reticulocyte count of >150,000
and patients suffering from severe disease. Hydroxyurea therapy was started at 15mg/kg/day
every 12 weeks up to maximum of 30mg/kg/day for a year. Haematological profile was done
along with liver and renal function test before starting the therapy and then was monitored
every 2 weekly. Result:During the period of 1 year 271 patients were examined. The
incidences of various types of crisis observed were as follows: painful crises 160, haemolytic
crisis 64, abdominal crisis 32, acute chest syndrome 3, CNS crisis 4, squestrationcirsis 5,
Aplastic crisis 2, hand foot syndrome 1. Out of 271 patients 103 received HU therapy. The
mean age of patients was 7.3 years. Sixty eight percent responded to a dose of HU up to
20mg/kg/day. There was significant reduction in VOCs from, number of days of
hospitalisation, transfusion and haemolysis from 2.6 ±1.2 to 0.9±0.4, 8.1±5.2 to 4.0±2.7,
6.4±6.6 to 3.3±4 and 3.2±1.2 to 1.2±0.7 per year respectively. There was significant increase
in Hb& HbF levels from 8.5±1.2 to 9.8±2.1 and 6.0±3.6 to 18.0±4.0 respectively. There was
significant reduction in ANC, WBC and reticulocyte count from 6.7±1.3 to 5.2±1.3, 13.3±5.2
to 8.4±2.7*103 and 3.1±1.3 to 1.5±0.77%. None of the child suffered from growth retardation
or leukemic, transformation; myelosuppression was seen in 6.8% and was reversible after t
cessation of therapy. Conclusion:There appears to be a beneficial role of HU therapy for the
prevention of anaemia and growth of children suffering from SCD. However more studies are
required to establish it’s role in SCD.
HO/3 (P) ACUTE PARAPLEGIA IN CHILDREN UNDERGOING ALL THERAPY
Leni G Mathew, Rikki John, Maya Thomas, Sridhar Gibikote
Ped-Hem-Onc, Dept of Child Health, Christian Medical College Hospital. Vellore, India
Email: [email protected]
Background: Neurological complications are well known in children undergoing treatment of
Acute Lymphoblastic Leukemia. These may be due to disease itself or complications arising
from treatment. We report six children who developed paraplegia while on treatment of ALL
from a tertiary care hospital in South India. Results: All six patients were boys with a mean
age of 6.8 years (2 – 13 years) at the time of diagnosis. Only one child had CNS disease at
diagnosis. All of them were treated on the modified MRC UK ALL protocol which consisted
of IT Methotrexate as prophylaxis for CNS Leukaemia. Neurological deficit developed
during maintenance phase in 4/6, reinduction in one and relapse protocol in one child. Onset
of paraplegia ranged from 1week to 5 weeks after IT methotrexate. All of them presented
with weakness in the lower limbs, 4 had urinary retention at presentation. In 5/6 CSF studies
were negative in all patients for CNS relapse and GuillianBarre syndrome. One child had
Acinetobacteriwoffii infection and associated with polyradiculoneuropathy. Nerve
conduction velocity studies in five of them showed evidence of motor
polyradiculoneuropathy. MRI scan of the spine showed leptomeningeal enhancement
especially in caudaequina region in four patients. Various therapeutic options including high
dose steroids, IVIG, folinic acid, Vitamin B12 and aminophylline were tried with no clear
benefit. Two of the six children progressed to develop flaccid quadriplegia with bulbar
weakness, diaphragmatic paralysis and subsequently succumbed to the illness. The remaining
four patients developed permanent sequelae. Conclusion: We conclude that serious
neurological sequelae in the form of paraparesis can develop following intrathecal
methotrexate injections during ALL therapy. There was no clear high risk factor identifiable
in our analysis for these neurological complications. Our analysis has also shown no clear
benefit of using steroids, IVIG or folinic acid. Most of the children had an adverse
neurological outcome.
HO/4 (P) EFFECT OF PREGNANCY INDUCED HYPERTENSION (PIH) ON
HEMATOLOGICAL PROFILE OF MOTHERSAND THEIR NEONATES.
Suhil Rafaliya, Sara Dhanawade,VinayakPatki
Dept. Of Pediatrics &Neonatology, Wanless Hospital, Miraj, Dist- Sangli, Maharashtra 416410
Email: [email protected]
Objective: To study the haematological profile of mothers with PIH and their neonates and
study the outcome these babies. Method: The present study was carried out as a prospective
case control study at the 8 bedded NICU over a period of one year. During this period a total
of 50 neonates admitted to NICUborn to mothers with PIH were studied and 50 babies born
to normotensive mothers born during same period served as controls. All the babies were
studied from the day of admission or birth and followed up till discharge or death.The effects
of maternal hypertension on the hematological profile of mothers & their neonates were
studied. Results: Majority (76%) of babies born to PIH mothers were preterms.They had
significantly higher incidence of thrombocytopenia(28% vs 0%)& nucleated RBCs(60% vs
18%).Higher MCV (113.55 ± 6.65 vs 107.01 ± 6.77) &MCH values (36.128 ± 2.19 vs34.018
± 2.34) and lower Absolute Neutrophil Count at 0 hr (6887 vs7998/mm3) & at 48 hrs (5859
vs 6876/mm3) were seen in them than controls. Though None of these cases were
polycythemicor had significant neutropenia, they had higher incidence of neonatal
sepsis(16% vs 0%). Conlusion: Babies of mothers with PIHhad high incidence of
thrombocytopenia, nRBC’s& neonatal sepsiswhile their mothers hadincreased incidence of
leucocytosis and thrombocytopenia.
HO/5 (P) A CASE OF INTRATHORACIC NEUROBLASTOMA
Hardik Gandhi, Abdulshamad, Varsha Shah, Bhairavi Shah
Department of pediatrics, Dhiraj Hospital, Pipariya, Gujarat
Email: [email protected]
Malignant tumour are not very common pathology in young child but some of them may do
manifest in early life. Neruoblastoma is one one them. Neuoblastoma arise from neural crest
cells which differentiate in cells of sympathetic ganglia and adrenal meulla. Neruoblstoma is
the most common extracranial solid tumour of childhood and most common neoplasm in first
year of life. They account for 7-10% of the childhood cancer. 90% are diagnosed under the
age of 5 year. 65% of neuroblastoma are found in retroperitoneum and other found in
posterior mediastenum, neck and pelvis. Case: one year male child was referred to dhiraj
hospital with complain of fever and breathlessness since 15 days which was treated as
pneumonia by private doctor and possibility of mediastinal mass on x-ray chest. The
investigation report from private showed Hb- 10.7 gm%, Total count- 21,100, differential
count- neutrophils-43, lymphocytes- 56, monocyte-01. Chest X-ray performed at private
hospital showed- Rt. Side lower zone consolidation with right mediasinal mass suggestive of
teratodermoid or thymic mass. After the child was admitted to dhiraj hospital further
evaluation of child done. Investigation showed Hb-11 gm%, total count-22,500 with
neutrophils-31, lymphocytes-60, monocytes-04, eosinophils-01. On further confirmation CT
Thorax was done, which showed- 7×6×4 cm heterogeneously enhanced mass in right side
post mediastinum with possibility of three diagnosis, 1)ganglionuroma 2) neutoblastoma
3)primitive neuroectodermal tumour with bilateral lower zone consolidation. To confirm the
diagnosis gun biopsy was performed under sonography visualization. Biopsy report showed
small, round cells with deeply staining nuclei and scanty cytoplasm with occasional rosette
with areas of hemorrhage and dispersible fibrillar necrosis suggestive of neuoblastoma.
Urinary VMA showed higher side of normal level. Then patient underwent for surgical
intervention by pediatric surgeons. After the surgical removal the specimen was sent for
thorough histopathology evaluation which confirms neuroblastoma. The character being 1)
according to Joshi and Silverman- neuroblastoma , poorly differentiated (grade -2 with low
mitotic rate) 2) according to Shimada- neuroblstoma – (stroma- poor neuroblstic tumour)
undifferentiated with low MKI ( <100 mitosis/ 5000 cells). The patient was referred to expert
oncologist for further management. He was started chemotherapy on standard protocol with
weekly cycle of cyclophosphamide and adriamycin given with total of 6 cycles. Patient was
thorough managed till he was discharged. Then child was followed on OPD basis and found
to be symptom free and on follow up x-ray done on completion of the treatment showed
complete resolution of that opacity. Still the patient is followed up in the OPD. Conclusion:
Thoracic neuroblasoma (TNBL) is a solid childhood tumor of neuroblast origin, which
presents in children most often in the first years of life. Treatment of TNBL is largely
directed by staging and the criteria for staging. Prognosis is dependent upon the stage of the
tumour. As the child was in stage-2, outcome was good.
HO/6 (P) A RARE CASE OF THALESSMIA WITH HEMOPHILIA( FACTOR 8
DEF.)
Jaikumar S Patel, Ashwini Kundalwal,Raghvendra Reddy
Room 313 New PG Hostel, SRTR Medical College & Hospital, Ambajogai, Beed,
Maharashtra
Email: [email protected]
This is a case report of a 6 yr old male child born of third degree consanginous marriage first
by birth order , brought by his father for complaints of fever since 5 days and generalised
weakness and increase paleness of body and swelling of left ankle.Patient had no history of
previous admission or trauma to left limb and fall or Koch contact.No history of any
bleeding manifestation in pastor family history of bleeding disorder was present.On
examination patient’s built was average,severe pallor was present with haemolytic
facies(maxillary bone prominence,depressed nasal bridge with enlarged skull) with huge
splenomegaly.Patient’s Hb electrophoresis was suggestive of Thalessmia and was transfused
with packed RBCs.For left ankle swelling X ray of ankle was done which was insignificant.
Blood investigations were done like RA factor,Uric acid,ESR and NSAIDS were given to
empirically treat rheumatic and rheumatoid arthritis.On aspiration of left ankle joint
hemorraghic fluid was collected. Clotting time and aPTT which were 8 mins and 48
seconds(control 34 seconds) respectively were both significantly prolonged with normal
Bleeding time and PT/INR.Blood sample was sent for factor 8 levels which came to be
midly decreased.(activity 9% ). On this basis diagnosis of Hemophilia was done in a patient
of Thalessmia Intermedia.Patient received Cryoprecipitate as Factor 8 Concentrates were not
available nearby. Swelling subsided in 4 days after transfusion.Patient since then has been
twice admitted for Hemoarthosis and relieved after 4 to 5 days of FFP tranfusion.
HO/7 (P) THE IMPACT OF DAILY VERSUS ALTERNATE DAY AND ONCE
WEEKLY IRON THERAPY ON HEMOGLOBIN RISE & IRON STATUS IN
CHILDREN AGED 6-14 YEARS, SUFFERING FROM IRON DEFICIENCY
ANEMIA
Krishan Kumar Aggarwal, M. S. Pannu, Karnail Singh, Amarjeet Singh
A-30 Madhav Puri, Maholi Road, Mathura
Email: [email protected]
Abstract - Iron deficiency Anemia (IDA) is the most prevalent nutritional disorder
worldwide, especially in developing countries. Different supplementation schemes to build
iron stores in 300 children aged 6-14 years were investigated. Children were divided into 3
groups of 100 each. We compared the efficacy of daily, alternate day and weekly iron
supplementation under supervised conditions in the treatment of mild and moderate anemia
[hemoglobin (Hb) 7.0–12.0 g/dl] in children studying in Government Elementary Schools of
rural Amritsar. The study was a cluster-randomized trial, participants were aware of the
treatment assigned. Children were administered a single dose of albendazole 400 mg at
enrollment followed by 3 months of iron supplementation daily in group I, 6 months of iron
supplementation thrice weekly in group II and 6 months of iron supplementation weekly in
group III. Dose was same in all groups i.e. 4-6mg/kg/day. Hb and serum ferritin (SF) levels
were significantly higher in daily group compared with alternate day group after 3 months.
But after 6 months, both levels were lower in daily group despite taking equal number of iron
tablets, though statistically significant difference was observed in Hb only. Rate of
disappearance of symptoms & signs of IDA in daily group was higher along with the
incidence of side effects and drop rates. Hb and SF levels were constantly increasing at
slower rate in weekly group. Though both levels were significantly less, compared with other
groups, but had the advantages of negligible side effects and drop rate after 6 months. This
study concludes that all treatment modalities are efficacious in the treatment of IDA.
Alternate day therapy appears to be superior to daily because of fewer side effects, lesser
drop rate, but with longer duration of treatment. Weekly supplementation may be
recommended only in situations where needed long compliance could be assured.
HO/8 (P) IMMUNOGLOBULIN THERAPY FOR AUTOIMMUNE NEONATAL
THROMBOCYTOPENIA
Pooja Kamath, Charusheela Warke, Sushma Malik, Ashwin Saboo
Neonatology Division, Department of Pediatrics, T.N Medical College, BYL Nair Hospital,
Mumbai
Email- [email protected]
Introduction: Autoimmune neonatal thrombocytopenia (AINT) is essentially, due to maternal
disease (idiopathic thrombocytopenia [ITP] or systemic lupus erythematosus [SLE]), which
manifests in the neonate as a result of the transplacental passage of maternally produced
autoantibodies directed against various glycoproteins on the platelet surface. Here we report a
rare case of AINT secondary to maternal idiopathic thrombocytopenia. Case Summary- A
second gravida mother delivered a full term 2.7 kg male child by LSCS done for
oligohydraminos and previous LSCS. Antenatally, the mother had got diagnosed as
idiopathic immune thrombocytopenia and was treated with steroids, platelet transfusion and
fresh frozen plasma. At birth, the neonate was clinically stable with no bleeding, rash or
petechiae. Routine investigations were essentially normal except for low platelet counts41,000/L. Serial platelet count remained low and when it was less than 30,000/L, the neonate
was treated with IVIG twice. Repeated USG done to rule out IVH remained normal. Patient
was discharged at 2 weeks; however the platelet counts at 8 weeks of follow up are yet low (
45,000/L). In the present case, diagnosis of autoimmune neonatal thrombocytopenia was
based on the finding of thrombocytopenia during the first 72 hours of life, evidence of
maternal ITP and absence of an alternate cause for the neonatal thrombocytopenic state like
PIH, SLE etc. Discussion Autoimmune thrombocytopenic purpura during pregnancy may be
complicated by fetal and neonatal thrombocytopenia in 10-50% cases and the risk for
intracranial bleeding or death has been estimated at 1·3%. Autoimmune thrombocytopenia in
affected infants is characterized by its early onset, asymptomatic baby, clinically stable and
normal physical examination. In rare instances intracranial haemorrhage can occur. All
neonates of mothers with AINT should have a cord blood platelet count determined at birth
and repeated daily till the seventh day before it rises spontaneously in most cases. If platelet
counts of < 30 × 109/l, it is advisable to treat neonates with IVIG / prednisolone regardless of
whether or not there is evidence of bleeding. Platelet transfusions may be useful as adjunct to
immunoglobulins in a few patients with severe haemorrhage. Conclusion: Transient
autoimmune thrombocytopenia may occur in infants born to mothers suffering from
autoimmune thrombocytopenic purpura. These neonates should be routinely screened for
platelet counts and for life threatening bleeds. Management for neonates with very low
platelet counts includes intravenous immunoglobulins according to prescribed guidelines.
HO/9 (P) CASE REPORT OF ACUTE MYELOID LUEKEMIA PRESENTING AS
BILATERAL PROPTOSIS AND CRANIAL NERVE PALSY
Jaikumar Patel, Manish Tiwari, Pallavi Saple.
Room 313 New PG Hostel, SRTR Medical College & Hospital, Ambajogai, Beed
Email: [email protected]
A 10 year old female child came with complains of bilateral proptosis with difficulty in
closing eyes , even while sleeping since three months, difficulty in swallowing and difficulty
in speech since last seven days .. On clinical examination vitals were stable, afebrile, mild
pallor, no lymphadenopathy, no bone tenderness, no petechaie and no obvious thyroid
swelling. There was bilateral proptosis with mild conjunctival congestionOn CNS
examination there was palsy of VII, IX and X cranial nerves of left side , which was lower
motor neuron type. Rest of CNS and other system were normal on examination. There was no
spleen palpable on clinical examination. Patient already had done thyroid profile, in which T3
was 84.2 nanogram/dl , T4 was 18.4 microgram/dl, TSH was 1.5 mIU/ L and TPO antibody
levels were 30.25 U/ ml . These findings were not consistent with hyperthyroidism, so
patient was investigated further in form of complete blood count, peripheral smear, USG B
scan of orbit and CT Brain with orbit with contrast. CBC showed normal RBC and Leucocyte
count, platelete count was 92,000/cumm , Haemoglobin was 9.2 g/dl. Peripheral smear was
normal except platelets were depleted . USG B scan of both orbits was normal. CT brain with
orbit revealed proptosis secondary to infiltration ? cause. Bone marrow examination was
done which showed altered myeloid to erythroid ratio secondary to myeloid hyperplasia, 60%
blast cells of myeloid series almost replacing marrow, erythroid series decreased in number
with normal in maturation and morphology, These findings were suggestive of Acute
myeloid leukemia.
HO/10 (P) COMPARATIVE STUDY OF CPK, 24 HOUR URINARY CREATININE
AND LDH ACTIVITY IN PATIENTS WITH AND WITHOUT VASO-OCCLUSIVE
CRISIS IN SICKLE CELL DISEASE ADMITTED IN WARD OF DR.BRAMH
S.Phuljhele, Amit Valbhani, P.Beck, Virendra Kurrey
Administrative Block, Department Of Pediatrics, Pt JNM Medical College & Dr Br
Ambedkar Memorial Hospital, Raipur, Chhatisgarh.
Email - [email protected]
Introduction-Previously it was thought that the onset of crisis in a sickle cell disease cannot
be predicted. But now it’s studied that most patient who suffer from painful crisis can be
aborted at an early stage. Aims and objectives :- To measure and compare CPK,LDH and 24
hour urinary creatinine levels in study group. Materials and methods:- This study was
conducted from November 2010 to August 2012.Patients between 3 years to 14 years were
included in this study. Cases were taken as patients of VOC and controls as those admitted
without VOC. CPK, LDH and 24 hr urinary creatinine were measured immediately after
sample collection in an automated IL analyser and levels were measured at 25 degree Celsius.
Exclusion criteria included cases with renal failure,critical cases in PICU, hepatic failure.
Results-150 patients were studied out of which 58 of VOC, 47 of hemolytic, 10 of
megaloblastic, 9 of sequestration and 5 of aplastic crisis and remainder 31 were admitted only
for blood transfusion. Pedictability of crisis was studied in relation to values of CPK,LDH
and 24hr urinary creatinine. CPK(56% cases) and LDH(75%cases) were more raised in VOC
as compared to hemolytic(0.02%,55%),sequestration(11%,o%), megaloblastic and aplastic
crisis whereas 24hr urinary creatinine gave equivocal results. Conclusion:- CPK,LDH and
24hr urinary creatinine are of potential value for monitoring the onset of tissue ischemia in
sickle cell crisis. Of the three, CPK has the highest sensitivity and 24 hr urinary creatinine has
the least sensitivity.
HO/11 (P) CRANIAL RADIOTHERAPY FOR ACUTE LYMPHOBLASTIC
LEUKEMIA: FAVORABLE OUTCOME WITHOUT ITS USE.
Faisal R Guru, Ravi Kumar Parihar, Akanksha Chichra, Amita Mahajan
Indraprastha Apollo Hospital, New Delhi
Email: [email protected]
Introduction: Cranial prophylaxis is an integral part of the treatment of acute lymphoblastic
leukemia (ALL). Cranial radiation (CRT) is no longer recommended in patients with standard
risk (SR) disease. However some centers continue to use CRT in the high risk (HR) group. At
our centre CRT is given only to those patients who present with central nervous system
(CNS) disease at diagnosis while others receive only intrathecal chemotherapy. Aims &
Objectives: To analyze the incidence of CNS relapse between the SR and HR patients with
ALL. Materials & Methods: We analyzed data analysis of all children with ALL aged 1 to 18
years treated at our center between 2003-2012 regarding CNS disease status at diagnosis,
treatment given for CNS prophylaxis, treatment for CNS positive cases and outcomes.
Results: Data of 163 patients revealed the mean and the median age to be 8.2 and 7.1 years
respectively. CNS disease at diagnosis was seen in only 5(3.08%) patients all of whom
received CRT. In addition 2 more patients received CRT because of inability to come for
regular follow up while all others received intrathecal chemotherapy alone. Out of 156
patients, 76 (48%) were SR and 80(52%) were HR. There were a total of 12 relapses, 8 bone
marrow, 1 testicular, 1 bone marrow plus CNS and 2 extramedullary relapse. Among the
relapses 7 (58.3%) were SR and 5(41.7%) were HR (p<0.05). The only CNS relapse was in
the SR group. Of the 7 patients who received CRT, 3 patients had CNS relapse. At a median
of four years from diagnosis, 145 (88%) are alive, free of disease. Conclusion: Optimal
outcomes can be achieved in patients with high risk ALL even without use of cranial
radiotherapy.
HO/12 (P) REPEATED EPISODE OF ASCITIS: DIAGNOSTIC DILEMMA
Neha Jobanputra, Vivek Shah, Sushma Save, Alpana Somale, Sandeep Bavdekar
Flat No 402, OMKAR CHS., 511/B R.P. Masani Rd., Matunga, Mumbai 400 019
[email protected]
Introduction: Neuroblastoma is the common extra cranial solid tumor of infancy and < 10%
remain undiagnosed till 7 years of age. Tuberculosis superimposed with neuroblastoma or
even vice versa is a rare entity. Here we are presenting such a baffling case. Case : 8 yr. old
female born of non-consanguineous marriage presented with distention of abdomen , pain
and recurrent pleural effusion since 4 months. On examination she had mild pallor and pedal
edema with gross ascites .On systemic examination revealed hepatomegaly (5cm with span
12cm).Other systems were normal. USG abdomen showed hepatomegaly ascites and pre and
Para aortic mesenteric lymph node enlargement ,CT thorax revealed bilateral pleural
effusion with right side consolidation. CT scan of Abdomen showed multiple enlarged
conglomerated and few calcified lymph nodes encasing the aorta and proximal coeliac artery
and portal vein and pancreas. Complete blood count and biochemical tests were normal.
Ascitic fluid examination showed Proteins 1.3 gm%, Cholesterol 34mg%, Microscopy:
180WBCS/mm3, 80 RBCS/mm3, on smear 98% lymphocytes .Pleural and ascitic fluid was
negative for AFB and malignant cells. Her gastric lavage was positive for acid fast bacilli for
which she was on 4Drug ATT for 4 months but recurrence of ascites and pleural effusion
garnered to revise the diagnosis. CT guided biopsy of retroperitoneal lymph node clinched
the diagnosis as neuroblastoma stage IV. Discussion: Neuroblastoma is one of the small,
blue, round cell tumors of childhood with prevalence of 1 case per 8000-10,000 children. It
has been called the great mimicker because of its myriad clinical presentations related to the
site of the primary tumor, metastatic disease, and its metabolic tumor by-products. Such a
similar involvement superimposed with a positive gastric lavage for AFB gave a double
diagnosis in our patient. Conclusion: In the Indian setting the prevalence of tuberculosis is so
wide often such a rare case of neuroblastoma would not be diagnosed if it had not been for
the prompt evaluation and action about the unresponsiveness to ATT.
HO/13 (P) RESTING HEART RATE IN ANAEMIC CHILDREN
Baljinder kaur, Jaswir singh, Sheenu, Neha
197 Sewak Colony, Patiala, Punjab
Email: [email protected]
Introduction: Anaemia is a common presentation hence there is a need of exploration. Aims
& Objectives: To study effect of Anaemia on Resting Heart Rate. Design: A Prospective
study. Material & Methods: 75 children age ranging from 1to10 years with complaints of
Anaemia visiting Paediatrics outdoor of Government Medical College& Rajindra Hospital
were the subjects of study. Children suffering from respiratory ailments were excluded.25
healthy children were taken as controls.For the purpose of study children were divided to
various groups of 1to <4 years ,>4 years to 6 years>6years to 10
years.Name,Age,Gender,Presenting complaints,General physical examination,Systemic
examination,Resting Heart Rate,Respiratory rate ,hemoglobin estimation &type of anaemia
were recorded on predesigned pre tested proforma data so obtained was analysed. Results:
40[53%] children were females while35[47%] were males.40[53%] were in the age group of
1to <4years,22[27%] were in age group of >6 to 10years.7 ,4,2 children were found to be
having haemoglobin less than 7 mg% in age group of 1to<4,>4to6years,>6to10years
respectively.Mean restingheart rates in children having Haemoglobin>7 to 10mg% were
116,100,90per minute in age group of 1to<4years,>4to6years,>6to10yearswhile in children
having Haemoglobin<7mg% Mean resting Heart rates were122,108,104per minute in
respective age groups.Mean resting heart rate in control group was 110,100,88per minute in
respective age group of 1to<4years,>4years to 6 years ,>6 years to 10 years respectively.
Conclusion: Resting Heart Rate remains normal in moderate anaemia
HO/14 (P) CLINICO-HEMATOLOGICAL SPECTRUM OF PNH IN PEDIATRIC
AGE GROUP
Prusty J.Bikrant kumar, Swain Arakhita, Mohanty Niranjan, Sethy Sudha, Jena R.K
Dept.Of Paediatric, S.C.B.MCH, Cuttack, ODISHA.
Email: [email protected]
Introduction- PNH is a rare disorder in children, it occurs as a consequence of non-malignant
clonal expansion of one or several hematopoetic stem cell, that have acquired somatic
mutation of PIGA gene. The progeny of affected cells are deficient in GPI-AP (GPI-anchor
protein) like CD55 & CD59, that accounts for intra vascular hemolysis and primary
manifestation of disease. Aim- To study the clinico-hematological spectrum of PNH in
pediatric age group. Materials And Methods - A retrospective study of 15 PNH patients from
6yrs-14yrs age group with mean age of 11.4yr in the period june2011 to july2012 were
studied in our pediatric department in collaboration with department of hematology.
Multicoloured flow-cytometric analysis of peripheral blood granulocytes for CD55 & CD59
were done in all patients & their percentage of expression with clinical correlation were used
for diagnosis and management. Of 15 patients , 11(73%) had both CD55 & CD59 expression
reduced. 4(27%) had only CD59 expression reduced and out of the 4 patients 1 had severe
manifestation of the disease & rest three had minor clinical manifestations. Anemia was
observed in 11(73%), sepsis in 4(27%), classical hemoglobinuria in 3(20%), GI symptoms &
jaundice in 3(20%), aplastic anemia and neurological symptoms only in 1(7%) patient of
study group. No thrombotic complication were noticed during the study. ConclusionReduced expression of CD55 & CD59 were directly correlated with the severity of the
disease. Reduction of CD59 found to have aggressive clinical course. Anemia was the
commonest clinical presentation. No thrombotic complications were observed in studied
group.
HO/15 (P) PROFILE OF BONE TUMORS IN A TERTIARY CARE CENTRE
Akanksha Chichra, Ravi Parihar, Amita Mahajan
Department of Pediatric Hematology-Oncology, Indraprastha Apollo Hospital, Delhi
Email: [email protected]
Introduction: Bone tumors comprise 7% of all pediatric malignancies. Increasingly the focus
is on limb salvage modalities for local treatment. Aims and objectives: To retrospectively
review the spectrum of bone tumors in children ≤ 18 years at our centre. Materials and
methods: Medical records of children ≤ 18 years diagnosed to have primary bone tumors over
a seven year period (2005-2012) were evaluated with respect to demographic profile,
treatment and outcome. Results: Fifty children ≤ 18 years were diagnosed to have bone
tumors during the study period with a median age of 13 years (range 2-8 years). Ewing
sarcomas comprised 25/50, Osteosarcoma 23/50 and one case each of synovial sarcoma and
malignant mesenchymal tumor arising in a rib. The median age at presentation for
Osteosarcoma and Ewing sarcoma was 15 and 13 years respectively. The commonest site of
Ewing sarcomas was femur 6/25 followed by pelvis. The most common site for
Osteosarcoma was femur 16/23 followed by humerus 4/23. Four tumors were metastatic at
presentation. In the Ewing sarcoma group 17 have completed therapy. Of these 7 patients
relapsed and the survival at a median of 3 years was 58%. In the Osteosarcoma group 19 have
completed therapy. Of these 4 patients relapsed and the survival at a median of 3 years was
79%. Of the 38 patients with extremity lesions, 24 patients underwent limb salvage surgery.
Conclusion: A significant proportion of pediatric bone tumors can be taken up for limb
salvage surgery. Patients with Ewing sarcoma who underwent surgery had a better outcome
than those who received radiotherapy alone.
HO/16 (P) ALPHA THALASSEMIA (HbH VARIANT)
Braja Sundar Swain, K.C Digal, D.K Behera, V Samal, N Mohanty
Dept. of paediatrics, SCB MCH Cuttack-753007, Orissa
Email: [email protected], [email protected]
Introduction: HbH is a rare disease with incidence being 1:15000, manifests as anaemia (711g/dl), mild splenomegaly & not requiring BT. But the presence of a nondeletional alphaglobin mutation result in more severe anemia ,increased hepatosplenomegaly & a much
severe clinical course. HbH Constant Spring is the most commom form. Case report: A 7yr
male child present with Fever, weakness, increasing pallor for 15days. O/e reveals
hepatosplenomegaly (liver-8cm, spleen-7cm) & cervical lymphadenopathy. He had h/o
hospitalisation for similar complain 9 month back & on investigation reveals:- Hb-3.6, Retic5%, CPS-Haemolytic feature ,HPLC-normal ,ANA-normal, G6PD-3.8u/gHb, BM Studyerythroid hyperplasia &micronormoblast reaction, MPICT-negative. Pt had taken 3units of
BT at that time. Now on repeat CBC profile reveals;- Hb-4.7,retic-15%,cps-hemolytic
feature, no atypical cells. So planned for DCT,sickling &Hb electrophoresis. DCT & sickling
both are negative but Hb electrophoresis reveals;-HbH-24.2%,HbA-74.8%,HbA2-1.0%.
Finally the case diagnosed as ALPHA THALASSEMIA (HbH variant) . Discussion:Although HPLC is a simple rapid reliable method for the detection haemoglobinopathies but
Hb electrophoresis may play a valuable role in difficult cases .
HO/17 (O) EFFECTIVENESS OF CASCADE SCREENING IN DETECTION OF
THALASSEMIA CARRIERS AND PREVENTION OF THALASSEMIA
Mahantesh Matti, Nijaguna, Shivananda
Indira Gandhi Institute of Child Health, Bengaluru
Email: [email protected]
Abstract: Introduction: Beta thalassemia is one of the most common hemoglobinopathy
inherited by autosomal recessive pattern. It has high prevalence in Karnataka. It is an
economical, social and psychological burden to the patient, family and health care set up.
Thalassemia is basically a preventable disease by screening the population at large, but
unfortunately there is no screening protocol and it is not cost effective to screen general
population. Aims And Objectives: Assessing the effectiveness of “cascade screening” in
identifying the Thalassemia carrier status. Materials And Method: Setting: Type: Prospective
study. Period: 1 year, 2010-2011. Place: Thalassemia day care centre at Indira Gandhi
Institute of Child Health, Bangalore. Method: Cascade screening is a method in which the
relatives of index case are screened for carrier state detection. In this study, second and third
degree relatives (n=138) of 20 beta-thalassemia major patients were chosen for the study,
after informed written consent
persons were screened for carrier status by Hb
electrophoresis. Results: Index cases of Thalassemia: 20. Second and third degree relatives of
index case: 138. Thalassemia carrier state detected in: 55(40%) out of 138. Male: 28. Female:
27. 3 pregnant women were carriers, CVS done showed 2 foetus were carriers, 1 was
thalassemia major and it was aborted. For every thalassemia patient studied, there were about
3 thalassemia carriers which is significantly high. Discussion: Carrier rate of Thalassemia is
about 1-3% in south India. High incidence of consanguineous marriages, along with high
carrier status leads to increased incidence of thalassemia major patients. The screening test
for general population to detect Thalassemia carrier status is not being done and is not cost
effective and not feasible also. Screening the relatives of thalassemia index case is important
step in bringing down the incidence by offering genetic counselling and prenatal diagnosis.
Any screening programme centred around family and relatives of index case is highly
yielding. Conclusion: Cascade screening of thalassemia is very effective in identifying the
carriers and yield is high which was 40% in our study was. This is cost effective and feasible,
plays vital role in detection of thalassemia carrier status, and finally helps to bring down the
incidence of thalassemia major patients.
HO/18 (P) SEVERE FACTOR X DEFICIENCY
Rathod.A.D , Narvekar.R , Bhardwaj.R, Mane.S
Prof Of Pediatrics, Bldg-1, Flat # 15, Sir JJ Hospital Campus, Byculla, Mumbai- 400008,
Maharashtra
Email: [email protected]
Abstract:- Clotting factor X, or Stuart-Prower factor, is a vitamin K–dependent serine
protease that serves as the first enzyme in the common pathway of thrombus formation.
Factor X deficiency is a bleeding disorder that can be inherited or acquired. This disorder is
one of the world's most rare factor deficiencies. Inherited factor X deficiency is autosomal
recessive;where as Acquired factor X deficiency can be caused by severe liver disease,
vitamin K deficiency, or anticoagulant drugs . We here present a case of 30 month old boy
full term born of 3rd degree consanguinousmairriage; 3rd by birth order who presented with
history of focal convulsion of right half of body since 2 month of age. There was no
significant antenatal history of drugs; child recievedvit K at birth; 1st two sibling were having
no setback but there was history of delayed cord fall .On examination vitals stable
,pallor+,CRT<3sec,CTA good,not following light, no social smile, asymmetric moro’s reflex
,decreased movements of right side with brisk reflexes ;noecchymosis.child was
unimmunized till date received only BCG and OPV.USG skull revealed large
intraparenchymal bleed in left posterior parietal and occipital lobe; CBC revealed
anaemiawithout thrombocytopenia; grossly deranged PT and APTT; stool routine positive for
occult blood. Child’s coagulation profile revealed severe factor X deficiency (<1%) with
raised thrombin time and normal fibrinogen levels.child was treated conservatively by VitK ;
given blood and FFP; anticonvulsants and parental counselling was done.
HO/19 (P) PORENCEPHALIC CYST IN A CHILD WITH HEMOPHILIA
Rajniti Prasad, O.P.Mishra, Narendra Bagri
Department of Pediatrics, Institute of Medical sciences, Banaras Hindu University, Varanasi221005, India
E-mail: [email protected]
Abstract: Introduction: Porencephalic cyst is the presence of cyst or cavities within the brain
that results from developmental defects or acquired lesions, including infarction of tissue.
Rarely does a porencephalic cyst develops as complication of hemophilia due to repeated
intracranial bleed. To our knowledge this one of the rare case of porencephaly resulting as a
complication of hemophilia A. Objective: a case report: Case Report: An 8 years male child
who is known case of hemophilia A presented with left hemarthrosis for last 7 days and was
managed symptomatically at home with ice compresses. Child developed severe headache
and multiple episodes of vomiting over the last one day. There is no history of trauma to
head, seizures, unconciousness. Examination revealed left knee hemarthrosis with flexion
deformity of the same joint. Central nervous system examination revealed bilateral brisk
reflexes, babinski reflex and macewan sign was positive. Based on history and examination
possibility of intracranial bleed was kept and cranial CT scan showed subdural bleed in right
frontoparietal, left frontal region, cystic encephalomalacia of left cerebral hemispheres
dilatation of left lateral ventricle. The child was managed symptomatically with
recombinant Factor VIII replacement to achieve 100% activity for 10 days. ICH is a leading
cause of morbidity and mortality in patients with haemophilia, with a higher prevalence in
younger patients.
HO/20 (P) TO STUDY THE OUTCOME OF TREATMENT OF PAEDIATRIC
ACUTE LYMPHOBLASTIC LEUKAEMIA USING MCP-841 PROTOCOL IN
GENERAL PAEDIATRIC WARD VERSUS SPECIALIZED PAEDIATRIC
ONCOLOGY UNIT.
Biswajit Mondal, Prabhabati Banerjee
C/o Dr Prabhabati Banerjee, Fe 363, Salt Lake, Sector Iii, Kolkata-700106, West Bengal,
India
Email: [email protected]
Introduction: Childhood cancer is an emerging problem in our country. There are many
studies regarding the outcome of treatment of paediatric ALL using MCP 841 protocol but
from eastern India there is paucity of work. Thus a study based on the outcome of treatment
with MCP 841 in the General Paediatric Ward versus specialized Paediatric Oncology Unit
would help us in further broaden our field and setting of treatment. Aims and Objectives: 1.
To study the outcome of treatment of Paediatric ALL in general paediatric ward. 2. To
compare it with national data (from specialized paediatric oncology unit). Methods:
Paediatric ALL patient receiving MCP 841 from general paediatric ward were enrolled in the
study they were followed up. High risk ALL group were excluded from the study. Only 95
low and medium risk patients were included from June 2009 to July 2011in the study. The
outcome was observed in terms of Induction death, alive and disease free, infections and
hemorrhage. Data was collected on Excel data sheet and calculated by relevant software.
RESULTS
No. of
patients
General
ward
(Medical
college)
Specialize
d
Paediatric
oncology
unit(Natio
nal data)
Induction
death
Alive &
disease
free
Infection
Haemorrh
age
others
95
8(8.42%)
61(64.2%)
16(16.8%)
6(6.31%)
4(4.21%)
623
54(8.8%)
349(56%)
78(12.6%)
70(11.3%)
72(11.5%)
P value= 0.9 (not significant)
Conclusion: There are significant number of patients who are alive and disease free using the
same protocol when used in the general paediatric ward and the results as compared with that
of paediatric oncology unit is nearly the same. This data is of clinical significance to us as it
would help us treat ALL patients in a general ward and thus treatment would be available
even in those settings where specialized paediatric oncology units are not available.
HO/21 (P) THE CLINICO-HAEMATOLOGICAL PROFILE AND OUTCOME OF
PATIENTS WITH HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS AT A
TERTIARY CARE PEDIATRIC HOSPITAL IN INDIA.
Bhavna Dhingra, Jagdish Chandra, Kirtisudha Misra, Sunita Sharma.
Department of Pediatrics, Kalawati Saran Children’s Hospital, Lady Hardinge Medical
College, New Delhi- 110001. INDIA
Email id: [email protected]
Abstract Text:With increasing awareness about its existence, the number of cases of
Haemophagocytic –lymphohistiocytosis (HLH) being diagnosed is increasing. Aim: To
describe the clinic-haematological profile and treatment outcome of patients diagnosed with
HLH at a tertiary care Pediatric Hospital in northern India. Materials and Methods:
Retrospective review of medical records of patients diagnosed with HLH from January to
April 2012 at a tertiary care Pediatric Hospital. Data recorded in a pre-designed proforma
and analysed using SPSS version 16.0. Results: eight patients diagnosed with HLH in the
four month study period, five females and three males. The median age at presentation was
five years. Fever, pancytopenia and splenomegaly were seen in all. Median Hb was 5.8
gm/dl, total lymphocyte count 2245/cu.mm and platelet count 21,000/cu.mm. The median
serum ferritin levels were 7762ng/ml and the median triglyceride levels at diagnosis were 410
mg/dl. Conjugated hyperbilirubinemia was observed in three patients. Haemophagocytosis
was observed in two cases. NK cell activity and perforin expression was performed in three
patients . One patient had Griscelli syndrome and was positive for RAB 27a mutation. One
patient each had HLH with enteric fever and visceral leishmaniasis. There were two patients
with acute lymphoblastic leukemia. Three children had neurological involvement. Three
children received the HLH- 94 protocol and three patients received only steroids. None of the
patients received a bone marrow transplant. One child died, one is lost to follow-up and the
other six are currently still on treatment. Conclusions: Clinicians need to be aware of the
existence and the clinical presentation of HLH to be able to recognise this potentially fatal
condition early and institute appropriate therapy so as to maximise benefit and to reduce the
mortality and morbidity.
HO/22 (P) POST-INDUCTION MORBIDITY IN CHILDREN (AGE 1 TO 18 YEARS)
TREATED FOR ACUTE LYMPHOBLASTIC LEUKEMIA: A RETROSPECTIVE
COHORT STUDY
Shruti Khurana, Bhavna Dhingra, Jagdish Chandra, Varinder singh, Harish Pemde.
Department of Pediatrics, KSCH, LHMC, New Delhi-110001.
Email id: [email protected]
Introduction: Acute Lymphoblastic Leukemia (ALL), is the single most common malignancy
in children. The cure rates have improved substantially in the developed world, the
developing world is still far behind. There is lack of data addressing the problems faced postinduction that may have an adverse effect on the outcome. Objectives: To study the morbidity
profile of the ALL patients post-induction and correlate the Outcome to their present
Nutritional and Socio-Economic Status. Methodology: An Observational, Retrospective
Cohort Study of 33 cases of ALL treated according to the institutional protocol at a tertiary
care hospital - pediatric hematology-oncology division. Results: Total 33 patients treated
under the protocol, 20 (60.6%) survived. 5 (15.2%) died in the post-remission period .The
causes of death were stroke, pyogenic meningitis, septic shock, Infective Enterocolitis and
Fungal/ P. jiroveci pneumonia. and 5 patients were lost to follow-up. Each patient received
approx. 90 weeks (89.45±47.8) of treatment. The median age was 5 years (M/F=5.6:1). Two
relapses after completion and one after two-year abandonment of therapy. There were a total
of 16 episodes of febrile neutropenia; 12 episodes of sepsis; 9, 4 and 2 patients had fungal
infections, Hepatitis B/C and tuberculosis respectively. 2 developed vincristine-induced
neurotoxicity. Conclusion: The condition of children undergoing chemotherapy for Acute
Lymphoblastic Leukemia in terms of morbidity, mortality and outcome are significantly
different from those in the developed countries due to different socioeconomic status, disease
biology, genetic characteristics, and treatment-related factors.Early recognition and treatment
of comorbidities is of paramount importance to help improve outcomes in children with ALL.
HO/23 (O) PRO-THROMBOTIC RISK FACTORS IN CHILDREN WITH
ARTERIAL AND VENOUS THROMBOSIS
Nita Radhakrishnan, Avinash Kumar Singh, Sabina Langer, Tulika Seth, Pravas Mishra,
ManoranjanMahapatra, RenuSaxena
Department of Haematology, All India Institute of Medical Sciences, New Delhi
Email: [email protected]
Introduction: Thrombosis is increasingly being diagnosed both in neonates and children.
Testing for genetic prothrombotic risk factors in children with thrombosis has been heavily
debated. We undertook this retrospective analysis to identify the prevalence of prothrombotic
risk factors in children referred to us with thrombosis. Methods: The present study was a
retrospective analysis of paediatric patients with arterial (AT) and venous thrombosis (VT)
referred to the Department of Haematology, AIIMS, New Delhi for pro-thrombotic workup.
Patients aged 1 month - 18 years who presented to our centre between January 2009 and
February 2010 were included. Patients whose samples were received during the acute stage of
thrombosis were excluded. Due to considerations of cost, investigations were done in a
stepwise manner. ProC®Global was used as an initial screening test followed by Protein C
(PC), Protein S (PS), APCR (activated protein C resistance) and AT (Antithrombin III)
assays. Molecular studies of Factor V Leiden and MTHFR C677T polymorphisms were
performed on a subset of patients with increased suspicion. Age based reference ranges were
usedfor protein C, S and AT levels. Prothrombin G20210A mutation was not performed in
any patient. Results: A total of 132 patients with AT and 72 patients with VT were evaluated.
Mean age of patients was 6.53  5.15 years and 9.29  5.88 years in those with ATand
VTrespectively. Out of 132 patients with AT, 116(87.8%) presented with cerebral thrombosis
and the rest had peripheral thrombosis. Most patients with VT (>50%) presented with an
intra-abdominal site of thrombus. 24% and 11% patients presented with DVT of limbs and
cerebral venous thrombosis respectively. Acquired risk factors observed in children with VT
included sepsis, meningitis, central venous catheter and nephrotic syndrome.Family history
was positive in 18% of patients with AT and 16.6% of those with VT. Comparison of prothrombotic risk factors in children with AT and VT is given in table 1.
Table 1:
Arterial thrombosis
Venous thrombosis
P value
Mean age (years)
0.024a
6.53  5.15
9.29  5.88
M:F ratio
1.35:1
1.18:1
Protein C deficiency
3.7%
8.3%
0.201b
Protein S deficiency
4.5%
2.8%
0.715b
Antithrombin deficiency
1.5%
5.6%
0.188b
Factor V Leiden (homo)
4.7%
0
Factor V Leiden (hetero) 37.5%
19%
a
b
Independent t test, Fisher’s exact test
Discussion: In patients with AT, protein C and S deficiency was seen only in 3-5%. Factor V
Leiden was isolated in almost 20% of patients tested, probably because it was testedonly in
those with recurrent stroke or positive family history. In patients with VT, 32% had an
acquired risk factor. The presence of at least one genetic pro-thrombotic risk factor was noted
in 13 (18%) of our VT patients. Persistent antiphospholipid antibodies although reported
widely were not seen in our study. The importance of confirming the persistence of
antiphospholipid antibodies is highlighted here as children commonly have transient
antibodies. Patients with history of recurrence and those with a significant family history
should definitively be evaluated thoroughly.The role of developmental haemostasis needs to
be considered while evaluating the results of these tests. In resource-limited situations, a
judicious approach, with emphasis on genetic factors that are locally prevalent, and the
detection of which has definite therapeutic implications should be preferred.
HO/24 (P) CLINICAL PROFILE OF CHILDREN WITH FACTOR X DEFICIENCY
Nita Radhakrishnan, Avinash Kumar Singh, Mrinalini Kotru, Tulika Seth, Pravas Mishra,
Manoranjan Mahapatra, Renu Saxena
Department of Haematology, All India Institute of Medical Sciences, New Delhi
Email: [email protected]
Introduction: Factor X is a vitamin K dependent plasma protein, the deficiency is rare and is
recessively inherited. Among the rare bleeding disorders, factor X deficiency has a severe
presentation, characterized by hematomas, hemarthrosis, gastrointestinal and central nervous
system bleeding. As Factor X concentrates are not available, fresh frozen plasma (FFP) is
commonly used for both treatment and prophylaxis. Methods: The present study was a
retrospective analysis of paediatric patients diagnosed with Factor X deficiency at the
Department of Haematology, All India Institute of Medical Sciences, New Delhi. Pediatric
patients aged < 18 years who presented to our centre between January 2007 and July 2012
were included in the analysis. Data available from the clinical records were used for
information regarding symptoms, family history and investigations performed. Diagnosis was
suspected on the basis of prolonged PT and PTT and confirmed by specific factor assay.
Secondary causes like Vitamin K deficiency bleeding and liver disease were excluded.
Statistical analysis was performed using SPSS version 16.0. Results: 12 children with Factor
X deficiency were diagnosed during this period at our center. Mean age at presentation was
1.83 years (Range: 0.5-17 years). Male: Female ratio was 2:1. Presenting symptoms included
easy bruising and skin bleeds in 58% cases, mucosal bleeding in 20%, prolonged bleeding
from cuts and wounds in 32%, large hematomas in 16%, hemarthrosis in 16% etc. One
patient presented with knee hematoma and pseudotumour of right thigh that was later
operated. Positive family history was present in only 2 patients. All patients had an
uneventful neonatal period. 7 patients had spontaneous major bleeding in the form of
hemarthrosis, large hematomas requiring hospitalization, CNS, GI bleeding and hematuria.
Grade 3 severity of bleeding was noted in 7 patients (58%) where as grade 2 was seen in the
rest of the patients. 10 patients had severe deficiency (Factor X level<1% of normal). 2
patients had mild deficiency out of which one patient had received FFP 3 weeks prior to the
testing, and has been advised to repeat the test. Bleeding severity correlated well with the
factor levels at diagnosis. FFP prophylaxis was advised to all patients with severe deficiency.
Prothrombin concentrates were tried in none. 3 patients have undergone surgery under FFP
cover. 6 patients are being actively followed up at our center and are on FFP prophylaxis.
Discussion: Factor X deficiency produces a variable bleeding tendency characterized by both
mucosal and deep bleeding. The diagnosis is often delayed, as this condition is not suspected.
Patients with severe deficiencies can present with life threatening bleeding episodes.
Prophylaxis with FFP is currently the only viable option for prevention of bleeding in patients
with severe deficiencies.
HO/25 (P) DISSEMINATED LANGERHAN CELLS HISTIOCYTOSIS PRESENTING
AS CHOLESTATIC JAUNDICE
Rohit Kapoor, Ratan Gupta, K.C.Aggarwal, Rakesh Yadav.
Institute-Vardhman Mahavir Medical College & Safdarjang Hospital New Delhi-110029
Email: [email protected];[email protected]
Abstract: A 2 year old male resident of Mathura (UP), presented with progressive abdominal
distention ,Right ear discharge and seborrhoeic dermatitis since 6 months ,Fever on and off 1 month, high grade, intermittent in nature. Yellow discoloration of body since 1 month,
associated yellow colored urine, passage of clay colored stool, generalized itching. On
general physical examination the patient was afebrile, vitals were stable,normal
anthopometry, scalp showed papules with whitish scales(Seborrheic dermatitis),Icterus was
present, ENT examination showed purulent discharge,from right ear ,non foul smelling,dull
TM with central perforation,On per abdominal examination, patient had a distended abdomen
,with visible veins on lower chest and abdomen. patient was had hepatosplenomegaly of
moderate grade. Rest systemic examination was within normal limits. Investigations showed
9.5gm%,TLC 10,700/cc,S.bilirubin direct fraction was 8.9mg% and ALP was
raised(4045U/L),viral markers were negative, Liver was increased in size,on USG abdomen
gall bladder was not visualized, CECT abdomen revealed periportal hypodensity extending
along portal radicles, CECT head showed lytic lesions with enhancing soft tissue noted in
parietal bone, CECT chest showed multiple centilobular nodues in b/l lung fields,and cystic
changes in few nodules in upper lobe ,Lytic lesions in Rt iliac crest,X ray skull showed
osteolytic lesions, Skin Biopsy findings confirmed LCH,S100 and CD1a marker positivity,
Bone marrow examination was normal. Conclusion: There is need to emphasize that LCH
can present as cholestatic jaundice as well.
HO/26 (P) PREVALENCE OF OBESITY AND DYSLIPIDEMIA IN CHILDHOOD
CANCER SURVIVORS
Kamalakshi G Bhat, Saravanan R, Soundarya M
Kasturba Medical College, Mangalore, Manipal University
Email: [email protected]
Introduction - Obesity and dyslipidemia are common late morbidities in cancer survivors. We
report on the prevalence of these in our patient population. Methods - Cross sectional study
on 50 survivors who had been off therapy for at least one year, in a tertiary care teaching
hospital. Anthropometric measurements and fasting lipid profile were done and details
regarding the diagnosis and treatment were recorded. Results - Majority of our survivors had
leukemia (78%), followed by lymphoma. Two thirds were boys and 75% belonged to 5 to 15
years group at the time of study. 33 children were diagnosed before the age of 6 years. The
median duration since completion of treatment was 4 years (range 1 to 8). Thirty two
survivors had normal BMI, 11 were underweight and 7 were overweight. Among the group of
30 who received cranial radiotherapy, 5 were overweight and no obesity noted. There was no
statistically significant association found between the risk factors like early age at diagnosis,
female sex, radiation exposure, steroid use, L asparaginase use and BMI category. Waist
height ratio of >0.5, an indicator of obesity was found in 3. Mean cholesterol level was
165.74mg/dl (range - 96 to 241). Raised cholesterol level was found in 12 of the 50 survivors.
There was no significant association between raised cholesterol and the risk factors like sex,
steroid and L asparaginase use. Conclusions – Unlike earlier studies, we did not find obesity
or dyslipidemia in our survivor population. The preexisting nutritional state, socioeconomic
status of the family, genetic and racial variations probably contribute to obesity and
dyslipidemia.
HO/27 (P) PREVALENCE OF ANAEMIA IN RURAL BOYS AND GIRLS OF
UDAIPUR: A STUDY
Himanshu Sharma, Manasvin Sareen, Abhishek Ojha, Suhel Abbas, Aditya Goyal, Nitesh
Kumar, Gaurav Kumar, Devendra Sareen, Vibha Chaudhary
C/o Dr. Devendra Sareen 27 F New Fatehpura Udaipur
Email: [email protected]
Summary: Introduction: Anaemia is a common problem of adolescent. The present study was
conducted in school going rural adolescents of Udaipur to look for prevalence of anaemia.
Material and Methods: In this study 260 boys and 280 girls between age of 10-19 years were
selected. They were subjected to physical examination to look for pallor and Hb estimation
was done . In boys 18(6.92%) were having pallor ,among girls 60(21.42%) were found to be
having pallor. While 7(2.69%) boys out of 235 were anaemic and among girls 29(10.35%)
were anaemic. Conclusion :
Hence, we conclude that prevalence of anaemia is alarming
among rural girls. Thus rural girls should be regularly taught about nutritive value of
common food items so that they can cope up with iron requirements, along with regular
health check up to early diagnose and treat anaemia.
HO/28 (P) MENINGO-ENCEPHALITIS IN A CASE OF SICKLE CELL ANEMIA
Manmeet Kaur Sodhi, Karuna Thapar
Sri Guru Ram Das Institute Of Medical Sciences And Research, Sri Amritsar, Punjab
Email: [email protected]
Case Report: A thirteen year old female child presented to us with pallor for the past few
months, it was progressively increasing.. The past history of patient revealed a similar
episode three years back. She was not hospitalized then, was given two aliquots of blood on
opd basis. Thereafter girl remained asymptomatic for next three years although the mother
noticed that she was pale throughout this period. On examination of Abdomen, massive
Hepato-spleenomegaly was also noted ( liver span 12cm and 6cm below costal margins in
mid clavicular line , spleen approximately 20 cms below costal margins crossing the
midline). C.N.S. examination was normal. The investigations were as follows :- PBF :Hemolytic Anemia, Hemoglobin Electrophoresis , Hb Sickle: 76.2%. The girl was diagnosed
to be having sickle cell anemia, with acute heamolysis. PBF picture showed marked
hemolysis with reticulocytosis with a positive Direct Coombs test. After six units of blood
transfusions, she gradually improved. After one month girl again presented to us with
complaints of headache followed by generalized tonic clonic seizures. At the time of
presentation girl had pale and icterus, vitals were normal. Abdomen was soft hepatomegally
was still present with a liver span of 14 cms. firm in consistency with rounded margins.
Spleen was also palpable 5 cm below costal margin firm in consistency. Precordium was
hyperactive with a flow murmur grade III, S1 and S2 was normally heard. The investigations
were as follows:- Hb – 9 gm/dl, TLC 15500, DLC 70/20/5/5, PLATELETS 1.5 LAKH, PBFPredominantly microcytic and hypochromic smear with no evidence of haemolysis. Cultures
- blood and urine were sterile. CSF - proteins 80, globulins –ve, sugar 61(103), cytology 2
cell (lymphocytes). Culture –ve, USG ABDOMEN- hepatomegally with massive
splenomegally with cholelithiasis . MRI - few hyperintensities are observed along with
normal ventricular ependyma on FLAIR images suggestive of encephalitis. Girl was started
on antimeningitic antibiotics, antivirals, antiepileptics and iv fluids. There was fall in
heamoglobin levels for which blood was transfused. She again developed heamaturia which
gradually resolved during the course. The symptomatology improved and she was discharged
on oral antiepileptics and steroids. The girl is on regular follow up till date. Discussion:
About 7% of the world’s population is carriers of some form of hemoglobin disorder, there
being about 270 million carriers of sickle cell anemia and thalassemia, worldwide. The
cumulative gene frequency of haemoglobinopathies in India is 4.2%. Sickle cell disease is
an genetically transmitted hemo-globinopathy. Males and females have equal preponderance
with the ratio being 1:1. Young children with Hb SS may have splenic enlargement
associated with their hemolytic disease, with progression to the syndrome of hypersplenism
accompanied by worsening anemia and sometimes thrombocytopenia. The girl also had
massive spleenomegaly with thrombocytopenia. Altered splenic function in young children
with sickle cell disease is a significant factor leading to their increased susceptibility to
meningitis, sepsis, and other serious infections, mainly caused by pneumococci and
Haemophilus influenza, which was the presenting feature for this admission. The girl also
had cholelithiasis which is also a known complication.
HO/29 (P) PANCYTOPENIA
IN CHILDREN: ETIOLOGICAL PROFILE IN
NORTH INDIA
Subhash Chandra
Flat # 7036 Crossing Infra, Plot # 7 Crossing Republic, Ghaziabad, Uttar Pradesh
Email: [email protected]; [email protected]
Abstract: Background & Objective: To determine the etiological profile of pancytopenia in
pediatric patients. Patients & Methods: Medical records review of a 33-month period (2007 to
2009) was done at a tertiary-care hospital in Greater Noida, UP. Clinical and hematological
data of all patients with pancytopenia (Hb<10g/dL, total leucocyte count < 4X109d/L, platelet
count <150X109/L) at presentation was analyzed. Patients on cytotoxic chemotherapy, those
developing pancytopenia during hospital stay, patients referred from other centers with
diagnosed hematological malignancies, and neonates were excluded. Results: Forty-two
children (mean age 8.26 years, range 8.5 mo-13 year, M:F::1:0.8) were included.
Megaloblastic anemia, aplastic anemia and infections were the commonest causes, being
responsible for 23.8%,14.3% and 45.2% of the cases, respectively. Bone-marrow aspiration
(BMA) was helpful in reaching a definitive diagnosis in 86% of those in whom sufficient
marrow tissue was retrieved for analysis. Aplastic anemia was the commonest reason for
failure of BMA in providing a diagnosis. Interpretation & Conclusions: Majority (69%) of the
causes of pancytopenia among pediatric patients in this region are easily treatable. There is a
need to be aware of such conditions and appropriate investigative modalities should be
undertaken for the same. Key words: Pancytopenia, Etiology, North India
HO/30 (P) BONE MARROW PROFILES IN CHILDREN WITH PANCYTOPENIAS
AT A TERTIARY CARE HOSPITAL : A CLINICO-ETIOLOGICAL STUDY
Shiv Rk Dubey, R P Singh, A K Arya
Department Of Pediatrics, GSVM Medical College, Kanpur, UP – 208002
Email: [email protected].
Abstract: Objectives : To study (1) the various etiologies and their clinical correlates, in
patients of pancytopenia (2) the bone marrow cytology in patients of pancytopenia. Design :
A cross-sectional, observational, hospital based study. Setting : A tertiary care pediatric
hospital. Methods : All (n=170) children in the age group 1-18 years, with pancytopenia ,
admitted in our hospital between January 2011 and august 2012. All subjects and their
attendants were interviewed thoroughly for eliciting history, indepth clinical examination of
the patients was done and all the patients were exposed to Bone Marrow Aspiration. Those
already diagnosed as leukemia or aplastic anemia, those giving negative consent for bone
marrow or not willing to get admitted, were excluded. Results : Megaloblastic anemia was
the commonest aetiology (47%) , followed by aplastic anemia (25.8%) and leukemia
(17.6%).Bone marrow was normal in 12 (7%) and malaria was the cause in 4 cases(2.3%).
Most common clinical presentation was pallor (81%), fever (68%), and petechial
haemorrhages (51%) and other features included hepatomegaly (44.8%), splenomegaly
(37.2%) and lymphadenopathy (22.5%). pancytopenia is defined as Hb <10 gm%, platelet
count <1 lac /cubic mm and TLC <4000 cells / cubic mm. Conclusion: Attempts should be
made to establish the aetiology of pancytopenia without delay. Easily treatable causes if
identified early can significantly decrease the mortality and morbidity of pancytopenic
children. Key-words: Pancytopenia , bone marrow aspiration, children , etiology.
HO/31 (O) HEMATOPOIETIC STEM CELL TRANSPLANT IN THALASSEMIA
MAJOR: SINGLE CENTER EXPERIENCE FROM INDIA
Gaurav Kharya, Dharma Ram Choudhary, Teena Sawhney, Rasika Setia, Anil Handoo,
Satyendra Katewa
Hemato-oncology & BMT, Dr B L Kapur Memorial Hospital, Delhi, India
Email:[email protected];[email protected]; [email protected]
om
Introduction: Thalassemia major (TM) is the commonest hemoglobinopathy occurring
worldwide. In India approximately 10,000 kids are born with TM each year. Lifelong red cell
transfusion along with timely initiation of iron chelation is the best way of managing these
patients. The downside of this treatment is huge cost. Aims & Objectives: An acceptable
curative alternative in a subset of patients is a matched sibling/related donor hematopoietic
stem cell transplant (HSCT). We present our experience of HSCT in TM. Methods: Case
records of all the patients who underwent HSCT for TM between Jan 2010 to Feb 2012 were
retrieved and findings were recorded in a pre-designed proforma. Results: A total of 34
children underwent HSCT in this duration. Mean age was 8.8 years (2–16 years). There were
19
males
and
15
females.
Twenty
six
underwent
transplant
using
Thiotepa+Treosulphan+Fludarabine
(Thio+Treo+
Flu)
conditioning
whereas
Busulfan+Cyclophosphamide+ATG (BU+CY+ATG) was used in 8 patients. Cyclosporine
and methotrexate were used for graft versus host disease prophylaxis in all the patients.
Thirteen were class II whereas 21 were class III. In 33 bone marrow was used as a source
whereas in 1 peripheral blood was used for harvesting stem cells. Mean follow up duration is
345.5 days (5-791 days). Mean nucleated cell dose was 6.4x108/kg (0.33-106.9x108/kg).
Mean CD34+ cell dose was 6.7x106/kg (1.82-14.02x106/kg). Mean day of neutrophil
engraftment was 17.2 days (12-54 days), three patients had not engrafted at the time of
evaluation. Mean neutrophil engraftment in Thio+Treo+Flu was 15.2 days whereas in BU +
CY + ATG was 22.3 days. Two patients (5.9%) had secondary graft rejection, both with BU
+ CY + ATG regimen. Four patients (11.8%) expired post transplant due to sepsis and other
transplant related complications. Overall survival was 88.2% whereas thalassemia free
survival was 82.3%. Conclusion: Hematopoietic stem cell transplant is an acceptable curative
treatment for management of TM in resource poor countries.
HO/32 (P) HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN CHILDREN IN A
TERTIARY CARE HOSPITAL - A CASE SERIES
Sruthi Gopinath, Sajitha Nair
MD Resident, Pediatrics, Amrita Institute Of Medical Sciences, Ponnekkara P O, Kochi,
Kerala, Pin 682041
Email: [email protected]: [email protected]
Introduction :Hemophagocytic Lymphohistiocytosis (HLH) is a rare macrophage related
hyper inflammatory disorder that presents as prolonged fever and a sepsis like syndrome.
Objective: To study the clinical profile, laboratory findings, management and outcome of
children with HLH admitted to a tertiary children’s hospital in India. Study Design:
Retrospective analysis of case records of all the children with a diagnosis of HLH from April
2009 to April 2012 Setting: Tertiary care children’s teaching hospital in India. Results: 25
cases met the inclusion criteria for the diagnosis of HLH based on the HLH-2004 protocol of
the Histiocyte Society. 52 % of the children were males. The mean age of presentation was 9
years.(range – 3 months to 17 years). The predominant presenting clinical features were fever
(96%), hepatomegaly (80%) splenomegaly(44%) and rash (36%) . CNS symptoms were
present in 68%.Anemia , neutropenia and thrombocytopenia were present in 76%, 52% and
68%respectively. Among the biochemical markers, hyperferritinemia was present in 88%,
hypofibrinogenemia in 16% and hypertriglyceridemia in 52%.Bone marrow examination
showed hemophagocytosis in 36% and lymph node biopsy was suggestive in 8 %. Systemic
onset JRA accounted for 44%, SLE for 32% and sepsis for 28% of cases. CMV was
identified in 8 %. Perforin gene mutation on screening analysis was positive in a 6 month old
infant with HLH.Corticosteroids were the most commonly used immunomodulatory agent
(80%) followed by cyclosporine in 40% and Etoposide in 12% .IVIg was used in 20%.
Overall survival rate was 80%. Conclusion: HLH should be considered in the differential
diagnosis of children with prolonged fever, organomegaly and cytopenia. Early recognition
and prompt therapy will improve the overall outcome.
HO/33 (O) QUALITY OF HEMOPHILIA TREATMENT IN INDIA- A SURVEY
Mohammed Ramzan, Satya Prakash Yadav, Veronique Dinand, Anupam Sachdeva
Department of Pediatrics, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi,
India
Email: [email protected]
Introduction:Hemophilia is a x-linked recessive disease characterise by recurrent bleeding
episodes especially in large joints. AIMS and objective: Hemophilia patients must receive
care from healthcare workers with expert knowledge of the bleeding disorder. This study
describes the epidemiology of hemophiliacs in India. Material and Methods: Survey was
done through organizing hemophilia camps in 12 cities of North India.A detailed survey form
was filled. Results: The survey included 403 patients with median age of 13 years (range 1.5 65 years). Hemophilia Aand B constituted 90.3% and 9.7% with severe, moderate and mild
in 70.5%, 22.3% and 7.2% respectively. At the time of diagnosis 22.3% patients received
factor replacement therapy, 61% received alternate form of treatment in the form of blood
transfusion, fresh frozen plasma, cryoprecipitate and 16.7% received minimal or no
treatment.The most common manifestation seen was target joint in 247/403 (61.3%) of
patients. Single target joint was involved in 215/403 (53.3%) which included knee joint in
43.7%, elbow 7.4%, hip 2%, shoulder 1.2% and ankle in 0.7%,. Twoand three target joints
were present in28/403 (6.9%) and 4/403 (1%) respectively. Clinical joint score (Child
instrument) and orthopedic joint score were pathetic in these patients. Permanent disability
was present in 155/403 (38.5%), of these 15.1% were using crutches. Inhibitor levels were
not checked in majority (>95%) of patients. Not more than 10 patients were covered with
insurance or reimbursement policy. Conclusion: This survey gives a snap view of health
services offered tohemophiliacs in India. More emphasis should be put on home care,
comprehensive services and prophylactic factor replacement to improve quality of life.
HO/34 (P) SAFETY AND EFFICACY OF DEFERASIROX IN THALASSEMIA
MAJOR PATIENTS
Virender Kumar Gehlawat, Pankaj Abrol, Veena Gahlot, Jyotsna Sen
Department of Pediatrics, Pt B D Sharma Postgraduate Institute of Medical Sciences, Rohtak,
Haryana, India-124001
Email: [email protected]
Abstract: Introduction: Deferasirox is FDA approved iron chelator. It removes iron from
reticuloendothelial tissue, parenchymal tissue and directly from myocardial cells. Literature
pertaining to safety and efficacy of deferasirox is limited in Indian setting. Aims and
objectives: To evaluate the safety and efficacy of deferasirox in thalassemia major patients.
Methods: Thirty patients of thalassemia major aged 3-18 years who received regular PCV
infusion in preceding 2 years, having persistent high serum ferritin (>1000 ng/dl) requiring
chelation with deferiprone were enrolled in the study. Children with active infection
(hepatitis B/C), chronic liver disease and chronic renal failure patients were excluded from
the study. Patients were started with deferasirox in the dose of 20 mg/kg/day for one month;
dose increased to 30 mg/kg/day in the absence of side effects and was continued subsequently
for one year. Serum ferritin (chemiluminiscent method), liver and renal function tests were
monitored. Slit lamp examination, fundoscopy, ECG and M- mode Echocardiography were
done at the beginning and completion of study. Serum ferritin level of cases in the preceding
6 months was taken as control. Results: Mean (SD) age of enrolled children was 10.9 (3.8)
years with male predominance [22 (73%)]. Of the total 30 patients, only 3 (10%) patients
were splenectomized. Proportion of children with malnutrition was 40% (n=12). Mean
number of transfusion requirement was 26.4 (4.7) with baseline serum ferritin levels of
5782.5 (2081.0) ng/dl. The observed mean serum ferritin were 5829.822146.93,
5654.382137.77, 5435.312125.43, 5208.392065.07 and 4982.362020.81 ng/dl at first,
3rd, 6th, 9th and 12th month of therapy respectively indicating that mean serum ferritin levels
significantly decreased after 6 months of deferasirox therapy (p<0.01). There were no
significant adverse events noted during the drug administration except for 2 patients
developing transient thrombocytopenia, one showed transient elevation in serum
transaminases and two showed transient rise in serum creatinine which reverted to normal
after omitting the drug for one month. There was no ocular, auditory or cardiovascular side
effects. Conclusion: Deferasirox results in significant reduction of serum ferritin levels within
6 months of initation of therapy and hence could be safe and effective alternate drug for
chelation in children with thalassemia major.
HO/35 (P) THE UNLUCKY XIII FOR A CLAN IN NORTH INDIA
Anand Kumar K, Satya Prakash Yadav, Vasant Chinnabhandar, Nivedita Dhingra, Jeevan
Garg, Sarita Verma, Aditya Kumar Gupta, Anupam Sachdeva
Division of Paediatric Hematology-Oncology and Bone Marrow Transplant Unit, Institute of
Child Health, Sir Ganga Ram Hospital, New Delhi.
Email: [email protected]
Introduction: Coagulation factor XIII deficiency is one of the rare bleeding disorders, the
most threatening and devastating complication is intracranial bleeding. The diagnosis can be
delayed as the standard coagulation profile is normal; it needs a high index of suspicion for
diagnosis.
Objectives: To describe the spectrum of bleeding manifestation in factor- XIII deficiency.
Although rare, with incidence of 1 in 3 million, here we describe 5 cases in a single clan with
consanguineous marriages. Failure to diagnose can lead to mortality by intracranial or bodycavity bleeds.
Method: Retrospective analysis of cases in a single clan.
Results: 5 cases of factor XIII deficiency were observed
C
S Age Age
Site of bleeding
Clot
% of Out
a
e
at
of
Solub
Factor -come
Others
S
x first diagn Umbil s.c/mu CNS
-ility
XIII
-ical
scle
e
blee -osis
test
level
bleed
Bleed
d
1.
F At
2M
+
+
+
Tooth
soluble 8.65% Doing well
birth
bleed
2.
F At
At
+
+
+
soluble No
Expired at
Sib of
birth birth
record age of
case 1
1 ½ year
3.
F At
3½
+
+
+
soluble 0%
Doing
birth year
Well
4.Sib
IntraExpired at
of
M At
At
+
+
+
abdomin soluble 1%
7 years
case 3
birth birth
al bleed
5
M At
At
birth birth
+
+
-
-
soluble
5%
Doing
Well
All 5 cases belong to the fourth generation of a clan that migrated from Pakistan with history
of consanguineous marriages. 3-females and 2-males, 100% had umbilical bleeding at birth
and subcutaneous / muscle bleeds, 80% had CNS bleeds, mean age of diagnosis was 0.7 yr,
clot was soluble in 5M urea in all cases, 5 cases had factor XIII level assay, 2 of them had
severe factor deficiency. 3 are doing well and 2 have expired. 1 died due to intracranial bleed
and another due to intra-abdominal bleed following trauma. Mean age of death was around 4
years. During the bleeding episodes they received fresh frozen plasma as treatment.
Conclusion: Any child with umbilical bleeding at birth should be investigated for factor-XIII
deficiency, especially if there is a history of consanguineous marriage. Care should be taken
to avoid trauma to head as it can cause more mortality and morbidity. With timely treatment
they can lead a long life.
HO/36 (P) LOW-DOSE RECOMBINANT URATE OXIDASE IN TUMOR LYSIS: A
SINGLE CENTER EXPERIENCE
Vasant Ranganath Chinnabhandar, Veronique Dinand, Satya Prakash Yadav, Dilraj K
Kahlon, Nivedita Dhingra, Anupam Sachdeva
Division of Pediatric Hematology- Oncology and Bone Marrow Transplant Unit, Sir Ganga
Ram Hospital, New Delhi, India
Email: [email protected]
Introduction: Uric acid, a primary culprit in TLS leads to ARF due to precipitation in renal
tubules at high serum concentrations. Manufacturer recommendations for recombinant urateoxidase (rasburicase) indicate doses of 0.15-0.2mg/kg for 5-7 days. Aims and objectives: To
compare outcomes in those not receiving rasburicase vis-à-vis those who did, and at lower
doses than currently recommended. Method: Children with laboratory and/or clinical features
of TLS from January-2006 to March-2012, were included. Outcomes, renal-replacement
therapy, rasburicase use and dosage were analysed. Result: Of 31 TLS cases, thirty (96.7%)
had hemato-lymphoid malignancies [ALL-17, Burkitt lymphoma (BL)-7, AML-4, other
NHL-2] and one had neuroblastoma. Only eight (25.8%) (ALL-4, BL-3 and NHL-1) received
rasburicase (due to financial constraints) of whom none required dialysis. Six (19.4%) of the
remaining 23 (74.2%) (BL-3, ALL-2, AML-1) patients required renal-replacement therapy
(dialysis). This difference did not achieve statistical significance (p=0.15), possibly due to
small patient numbers. Three (9.7%) who did not receive rasburicase died but all those who
received rasburicase are alive. Five of 8 patients who received rasburicase had ongoing TLS
at admission and 2 developed TLS following chemotherapy. Median pre-therapy uric acid
was 8.4mg/dL and median rasburicase dose was 0.15mg/kg. Median uric acid level post1st dose was 1.8mg/dL and 5 patients required only a single dose. Median cumulative dose
was 0.2mg/kg/patient. Conclusion: Rasburicase effectively prevents TLS-associated renal
failure, obviates dialysis requirements and associated complications. It is effective in TLS
even at doses lower than recommended. Low-dose therapy in resource-limited settings with
assessment of response-to-therapy and repeat doses, if required, can minimize costs.
HO/37 (P) PROMISING OUTCOME WITH VINCRISTINE AND STEROIDS FOR
KASABACH-MERRITT PHENOMENON IN A CHILD WITH KLIPPEL
TRENAUNAY SYNDROME
Sarita Verma P, Satya P Yadav, Vasant Chinnabhandar, Nivedita Dhingra, Anupam
Sachdeva
Division of Pediatric Hematology Oncology and Bone Marrow Transplant unit, Institute Of
Child Health, Sir Ganga Ram Hospital, New Delhi
Email: [email protected]
Background: Kasabach-Merritt phenomenon of consumption coagulopathy is well-described
in giant hemangiomas and may be life-threatening. Klippel-Trenaunay syndrome (KTS) is a
rare congenital vascular disorder comprising the triad of hemangiomas, bone and soft tissue
hypertrophy and vascular malformations. There are only a few case reports regarding
pharmacological management of this complication and none have reported an association
with KTS. Case: We describe a 16-year old male presenting with progressively increasing left
lower limb size with skin discoloration and thickening since 3 months of age. Swelling had
progressed gradually to involve the entire limb, scrotum and left side of abdomen. There was
intermittent but significant oozing of blood from scrotum and surrounding skin since 4 years
requiring repeated blood transfusions. He was referred for further management of oozing.
Appropriate blood components were given for low hemoglobin, thrombocytopenia and
markedly elevated d-dimer. Treatment with oral prednisolone (2 mg/kg/day) and weekly
vincristine (1.5 mg/m2) was initiated. Three weeks after initiation limb girth showed marked
decrease with no bleeding. Treatment was continued for 6 weeks. During this period he had
no oozing and limb size reduced. Unfortunately, he then developed herpes zoster on his
abdomen and hence treatment was withheld for next 6 weeks. Therapy for zoster was given.
Four weeks after stopping vincristine skin bleeding recurred and was controlled with FFP
support. Therapy was restarted post-zoster and given for another 6 weeks. He is doing well
presently. Message: Steroids and vincristine are a ray of hope in Kasabach-Merritt
phenomenon and especially appropriate when local measures are of no help or
contraindicated as in KTS.
HO/38 (P) PEDIATRIC LYMPHOMAS: A SINGLE CENTER EXPERIENCE
Vasant Ranganath Chinnabhandar, Satya Prakash Yadav, Veronique Dinand, Vikas Dua,
Nivedita Dhingra, Anupam Sachdeva
Department of Pediatric Hematology-Oncology and Bone Marrow Transplant Unit, Sir
Ganga Ram Hospital, New Delhi, India
Email: [email protected]
Introduction: Current therapy has improved survival in pediatric Hodgkin lymphoma (HL)
and non-Hodgkin lymphoma (NHL) to >75%. Aims and objectives: To analyse data on
pediatric lymphomas from our centre. Method: Diagnosis, treatment and outcomes in
histology-proven lymphomas from January-2005 to February-2012 were retrospectively
analyzed. Result: Seventy-nine patients [36 (45.6%) HL, 43 (54.4%) NHL] were identified.
Among 36 (45.6%) completing therapy, 34 (43%) were well. Twenty-five (31.6%) lost to
follow-up (LTFU), 15 (18.9%) died and 4 (5.1%) were on-therapy. Of six (7.6%) relapses,
one (HL) was cured; another (NHL) was on-therapy; 4 expired. Among HL, nodular
lymphocyte-predominant HL (NLPHL) was seen in 2 (5.5%) (Stage III-1 and IV-1), with one
currently on-therapy and another LTFU. Of twenty-one (58.3%) mixed-cellularity HL
(MCHL) (Stage-I/II/III/IV- 3/6/6/6), 13 (Stage-I/II/III/IV- 2/4/3/4) were well post-therapy, 4
LTFU, 1 relapsed (Stage IV) and 2 died. Among 11 (30.5%) nodular sclerosis HL (NSHL)
[(Stage-I/II/III/IV- 1/2/0/5); 2-not staged], 7 (Stage-I/II/III/IV- 0/3/0/4) are well posttreatment, 3 were LTFU and one child is on-therapy. LRHL and LDHL were not detected. In
NHL, 20 (46.5%) NHL-LL were diagnosed (Stage-I/II/III/IV- 0/7/9/4), 6 were well posttreatment (Stage-I/II/III/IV- 0/3/1/2), 7 LTFU and 7 died (3 with relapse). Of 12 (27.9%)
Burkitt lymphomas (Stage-I/II/III/IV- 0/2/4/6), 5 (Stage-I/II/III/IV- 0/2/1/2) were well posttherapy, one was on-treatment, 2 LTFU and 4 expired. Seven (16.3%) DLBCL were
identified [(Stage-I/II/III/IV- 0/2/1/3); 1-not staged], 2 (Stage-II) were healthy post-treatment,
2 LTFU and 3 died (relapse-1). Both (4.7%) (Stage-II/III- 1/1) ALCL cases LTFU.
Conclusion: Pediatric lymphomas, even higher-stage, are eminently treatable in the
developing world. LTFU remains a hurdle to improving outcomes.
HO/39 (P) LEIOMYOSARCOMA OF INFERIOR VENA CAVA IN PEDIATRIC
AGE- RAREST OF THE RARE
Anand Kumar K, Satya Prakash Yadav, Vasant Chinnabhandar, Nivedita Dhingra, Jeevan
Garg, Sarita Verma, Aditya Kumar Gupta, Anupam Sachdeva
Division of Paediatric Hematology-Oncology and Bone Marrow Transplant Unit, Institute of
Child Health, Sir Ganga Ram Hospital, New Delhi
Email: [email protected]
Objectives: To describe first ever known case of leiomyosarcoma of inferior vena cava in
pediatric age, up till now only 218 cases in the adults have been reported worldwide. Case
report of a patient admitted at our centre in August 2012. 13 year old boy presented with
polycythemia, jaundice, dyspnea and right sided abdominal pain. Chest x-ray was done in
view of dyspnea which showed retrocaval shadow. On 2D ECHO mass abutting on the right
atrium pushing the heart superiorly was seen. CT-chest revealed a large 14.2*10.7cm
supradiaphragmatic mass lesion invading the IVC superiorly abutting the right atrium and
inferiorly depressing the diaphragm with no pulmonary metastasis. CT angiography
confirmed it and also revealed hepatic venous obstruction. The tumor was resected
completely by a team of cardiac and hepatobiliary surgeons. He survived an 18-hour long
surgery and after intensive care management for a couple days was shifted to ward. The
histopathology report suggested a malignant mesenchymal tumor consistent with
leiomyosarcoma of IVC/right atrium. The final diagnosis was leiomyosarcoma of IVC of
segment III with subacute Budd-Chiari syndrome. The follow-up imaging has shown minimal
residual tumor. He is due for PET scan for staging and anthracycline based chemotherapy.
Conclusion: Leiomyosarcoma of IVC has not yet been described in pediatric age. IVC
sarcomas have a poor prognosis, but early diagnosis can lead to prolonged survival. Rare
tumors often need extensive evaluation and good multi-disciplinary care for maximizing
outcomes
HO/40 (P) SUCCESSFUL TREATMENT OF JUVENILE MYELOMONOCYTIC
LEUKEMIAWITH DOUBLE UMBILICAL CORD BLOOD TRANSPLANTATION:
A CASE REPORT
Nivedita Dhingra, SatyaPrakash Yadav, Vasant Chinnabhandar, Anupam Sachdeva
Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Sir Ganga Ram Hospital,
New Delhi, India
Email: [email protected]
Purpose: Juvenile myelomonocytic leukemia (JMML) is a rare pediatric malignancy with an
indolent course but high mortality. Hematopoietic stem cell transplant (HSCT) is the only
curative treatment. Umbilical cord blood transplantation (UCBT) is a promising alternative in
cases without a matched family donor (MFD). Aims and Objectives: Here we describe the
outcome of double UCBT in a case of JMML diagnosed in April 2010. Methods:
Retrospective review of case records. Case –An 8-month old boy, presented with pallor and
massive spleno-hepatomegaly and a peripheral blood picture suggestive of JMML. Bone
marrow morphology showed 8% blasts with immature myeloid and monocytic precursors.
Monosomy-7 was detected on cytogenetic analysis with no evidence of bcr-abl. JMML was
diagnosed and interim therapy with Hydroxyurea showed a partial response. A splenectomy
was later performed in view of progressive splenic enlargement and hypersplenism. A double
UCBT with cord-units that were 4/6 HLA-match was performed as MFD was unavailable.
Neutrophil engraftment occurred on d+15 post-transplant. The further course was
complicated by development of chronic skin graft versus host disease (GvHD) for which
steroids and imatinib were administered. Peripheral blood chimerism analysis on d+21
showed 100% donor cells (50% cord-1 and 50% cord-2). Presently the child is 300 days posttransplant with sustained remission and donor stem cell engraftment (64% cord-1 and 36%
cord-2) and mild chronic skin GvHD. Conclusion: Although JMML is a rare disease, it must
be considered in children with massive splenomegaly and monocytosis. UCBT is a suitable
alternative to MFDs for definitive therapy of this otherwise uniformly fatal disorder.
HO/41 (P) PROFILE OF GRAM-NEGATIVE BACTEREMIA IN A PEDIATRIC
ONCOLOGY UNIT
Vasant Ranganath Chinnabhandar, Satya Prakash Yadav, Chand Wattal, Nivedita Dhingra,
Ushma Singh, Mohammad Ramzan, Anupam Sachdeva
Department of Pediatric Hematology- Oncology and Bone Marrow Transplant Unit, Sir
Ganga Ram Hospital, New Delhi, India
Email: [email protected]
Introduction: Chemotherapy-induced immunosuppression, malignancy and treatmentassociated compromise of dermal and mucosal natural barriers are prime factors contributing
to increased sepsis-related morbidity and mortality in pediatric oncology. Gram-negative
organisms are frequently isolated from these patients. Intensive-care and increased antibiotic
use escalates risk of bacteremia and infections with multi-drug resistant organisms. Aims and
objectives: To study Gram-negative bacteremia, antibiotic sensitivity in isolated organisms
and outcomes in pediatric oncology. Method: We retrospectively analysed data of patients
(January 2010 to March 2012) with documented Gram-negative bacteremia and febrile
neutropenia also reviewed antibiotic-sensitivity of organisms and temporally traced patient
outcomes. Result: 28 independent episodes fulfilling above criteria were identified. The
commonest organisms were Pseudomonas (21.4%), E.coli (17.8%), Klebsiella (17.8%) and
Acinetobacter (14.3%). Organisms in six (21.4%) episodes were sensitive only to salvage
antibiotics like Colistin. S. maltophila, A. xylosoxidans, S. typhi, C. freundii, S. paucimobilis
were the other pathogens isolated in one case each (3.57%). Bacteria in eight (28.6%)
episodes produced enzymes such as ESBL and carbapenemase which contribute to antibiotic
resistance. Twelve (42.8%) patients had previously inserted PICC or CVL at the time of
bacteremia. Eight (28.6%) children died and bacteremia contributed to mortality. Four had
bacteremia sensitive only to Colistin. Carbapenemase-positive bacteria were isolated in 3
cases and ESBL-producing organisms in one patient. Conclusion: Gram-negative bacteremia
is a significant contributor to morbidity and mortality in pediatric oncology. Indwelling
venous access, repeated antibiotic use, nosocomial multiple-antibiotic resistant organisms and
therapy-related immunosuppression amplify risk of bacteremia in these children.
Identification of organisms and use of culture-appropriate antibiotics is fundamental to
minimizing mortality in these patients.
HO/42 (P) FATAL CYTOMEGALOVIRUS MYOCARDITIS IN A CHILD WITH
ACUTE LYMPHOBLASTIC LEUKEMIA
Sarita Verma P, Satya P Yadav, Vasant Chinnabhandar, Nivedita Dhingra, Anupam
Sachdeva
Division of Pediatric Hematology Oncology and Bone Marrow Transplant unit, Institute Of
Child Health, Sir Ganga Ram Hospital, New Delhi
Email: [email protected]
Objective: Cytomegalovirus (CMV) is an important and common cause of mortality and
morbidity in immunocompromised patients but has been only rarely reported in ALL as a
cause of myocarditis. We highlight a case of CMV myocarditis in ALL in remission that
ultimately proved fatal. Result: A two-year old boy with precursor B-cell ALL, diagnosed at
15 months, on final maintenance as per BFM high-risk protocol presented with blood-stained
mucoid stools with fever. Investigations revealed a highly resistant strain of Shigella sonnei
in stool culture which responded to antibiotics. Ten days post-hospitalization same symptoms
recurred. CMV copy numbers at this time were positive (38,300/μL) hence Valgancyclovir
was started but symptoms persisted with increasing CMV copy numbers (5 lac/μL). On 20th
day of hospitalization he developed congestive cardiac failure and was shifted to PICU.
Echocardiography showed severe left ventricular dysfunction with LVEF of 10-15%.
Mechanical ventilation and inotropic support was required. His CMV copy numbers
remained high and a possibility of CMV myocarditis was considered. He was managed with
IVIG and gancyclovir. Gradually his cardiac function improved and he was shifted back to
the ward and supportive care continued. He improved transiently but his CMV copy numbers
again increased and he became pancytopenic requiring frequent blood component support.
His diarrhea also worsened. Blood and stool cultures were persistently negative. He
developed features of refractory shock and subsequently succumbed despite intensive
resuscitation. Conclusion: CMV myocarditis, highly under-diagnosed and under-reported, is
one of the leading causes of fatal myocarditis. Timely diagnosis and escalation of CMVdirected treatment can help save such lives.
HO/43 (P) CYTOMEGALOVIRUS IN PEDIATRICHEMATOLOGY AND
ONCOLOGY: THE ENEMY WITHIN
Nivedita Dhingra, Satya Prakash Yadav, Vasant Chinnabhandar, Sangmitra Dutta, Chand
Wattal and Anupam Sachdeva
Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Sir Ganga Ram Hospital,
New Delhi, India
Email: [email protected]
S
n
o.
1
2
3
4
5
6
Purpose: Seroprevalence of cytomegalovirus (CMV) in developing nations is above 90%. Its
potential for re-activation poses a threat in the immunocompromised. Aims and Objectives:
To describe our experience with CMV in a Pediatric Hematology-Oncology unit. Methods:
Retrospective review of medical records of patients who developed symptomatic CMV
infection. The diagnosis of CMV was established by Real-time quantitative polymerase chain
reaction (PCR). Results: We identified 6 patients in our database diagnosed with CMV
infection between 2009 and 2012 with a median age of 9.5 years and a male to female ratio of
1:2. The clinical details are summarised in Table-1. The most common presentation was
pneumonitis, severe enough to warrant high-frequency ventilation in 2 cases. Other severe
event was fatal myocarditis in one case. The overall mortality from progressive CMV
infection was 50%.
Age Se Primary diagnosis
Clinical
CMV
CoTreatment
Outcome
x
features
copy
infection
numbers(
/mm3)
2.5 y M Acute
Enteritis+
>5 lacs
Shigellaso Gancyclovir X Expired
lymphoblastic
Myocarditis
nnei
28 days
leukemia (ALL)
8y
F
Refractory T cell HLH
>5lacs
None
GancyclovirX Alive
ALL
14
days
followed
by
Valgancyclovir
11 y F
ALL
Pneumonia ~2 lacs
None
GancyclovirX Expired
5 days
12y F
Immune
Pneumonia ~4 lacs
None
Gancyclovir X Expired
thrombocytopenia
14 days
7y
M Aplastic Anemia
Enteritis
~2500
S.
Valgancyclovir Expiredfro
maltophila x 7 days
m sepsis
15y F
Acute
myeloid Pneumonia 3600
Candidal
Gancyclovir X Relapse
leukemia
liver
7
days related
abscess
followed
by mortality
Valgancyclovir
Conclusion: CMV infections are often self-limited in immune-competent but feared greatly in
hematology-oncology units owing to their high mortality despite aggressive therapy. A high
index of suspicion is essential for timely diagnosis and therapy which may be life-saving.
HO/44 (P) CENTRAL NERVOUS SYSTEM COMPLICATIONS IN PEDIATRIC
HEMATOPOIETIC STEM CELL TRANSPLANTS: A SINGLE CENTRE
EXPERIENCE
Nivedita Dhingra, Satya Prakash Yadav, Vasant Chinnabhandar, RK Sabharwal and Anupam
Sachdeva
Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Sir Ganga Ram Hospital,
New Delhi, India
Email: [email protected]
Purpose: Children undergoing hematopoietic stem cell transplants (HSCT) are at risk for
multiple complications secondary to their immune-deficient states, drug toxicities and
interactions. Central nervous system (CNS) complications may be perplexing with potentially
life-threatening consequences in this cohort of patients. Aims and Objectives: To describe the
CNS complications in children who underwent HSCT at our centre between 2005 and 2012
focussing on clinical presentation, etiology and outcome. Methods: Retrospective review of
the transplant data base. Children with pre-existing neurological comorbidities were
excluded. Neurological assessment was performed by an experienced pediatric neurologist.
Results: Of 48 transplants (33 allogeneic and 15 autologous) acute CNS events were
observed in 8 patients (16.6%). The median age was 6.6 years (range 2 to 14 years) with
male: female ratio of 3:1. Seizures were the commonest symptom seen in 7 cases (87.5%)
followed by alteration of sensorium and transient cortical blindness. One patient presented
with status epilepticus requiring mechanical ventilation. The etiology was identified as
posterior reversible encephalopathy syndrome (PRES) in 4 patients (50%) based on typical
clinical and radiological findings, levofloxacin-induced neurotoxicity in 1 (12.5%),
intracranial infections in 2 (25%; fungal vasculitis =1 and Candida glabrata meningitis =1),
hyponatremic seizures secondary to drug interactions in 1 (12.5%).The patient with candidal
meningitis developed hydrocephalus which required neurosurgical intervention.Mortality
attributed to CNS complications was 4.1% as 2 patients expired from progressive
neurological deterioration. Conclusions: CNS complications in the post-transplant period are
significant and not uncommon. Clinical anticipation, close monitoring of blood pressure,
electrolytes, blood counts and relevant drug levels with decisive interventions are crucial in
maximizing survival.
HO/45 (P) CENTRAL NERVOUS SYSTEM COMPLICATIONS IN CHILDREN
UNDERGOING TREATMENT FOR HAEMATOLOGICAL MALIGNANCIES: A
DEVELOPING WORLD EXPERIENCE
Nivedita Dhingra, Satya Prakash Yadav, Vasant Chinnabhandar, RK Sabharwal and Anupam
Sachdeva
Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Sir Ganga Ram Hospital,
New Delhi, India
Email: [email protected]
Introduction: Survival of children with haematological malignancies in developing countries
remains suboptimal. It can be improved further by properly managing treatment related
complications. Central nervous system (CNS) complications are known to occur in these
patients and can be fatal. Aims and objectives: To analyse the CNS complications in children
undergoing treatment for hematological malignancies. Methods: Records of children
diagnosed with haematological malignancies between 2005 and 2012 were retrospectively
analysed. Exclusion criteria were leukemic infiltration of brain, radiotherapy-induced CNS
changes and pre-existing CNS disorder. Results: Of 403 pediatric haematological
malignancies we identified 27 patients with 28 neurological complications (6.9%). These
included posterior reversible encephalopathy syndrome (PRES, n=3), stroke (n=11; sagittal
sinus thrombosis = 4 and infarcts=7), intracranial infections (n=4; Enterococcus faecium
meningitis =1, neurocysticercosis=2 and fungal vasculitis=1), intracranial haemorrhage (ICH,
n=3), pseudotumor cerebri (n=2), SIADH (n=1), abdominal epilepsy (n=1), methotrexate
neurotoxicity (n=1), unclassified (n=2). All patients with PRES had typical radiological
findings and complete recovery. All cases of ICH were acute myeloid leukemia in DIC and
expired. SIADH developed in 1 child of Juvenile Myelomonocytic leukemia post- unrelated
cord-blood transplant secondary to drug interactions. Abdominal epilepsy was diagnosed
after EEG in 1 child with persistent abdominal pain who had previously presented with
seizures. Seizures were the commonest presentation seen in 85% of patients and 17.3% had
recurrent episodes in follow-up. Mortality from progressive CNS deterioration was 17.3%
(n=5). Conclusion: Neurological complications lead to morbidity and mortality in children
with haematological malignancies. Prompt recognition and intervention maximize favourable
outcomes. Long-term follow-up is essential to exclude persistent neurological sequel.
HO/46 (P) CASE SERIES OF STAPHYLOCOCCUS AUREUS INFECTION IN
PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA AT A SINGLE CENTRE
Anand Kumar K, Nivedita Dhingra, Vasant Chinnabhandar, Satya P Yadav, Anupam
Sachdeva
Division of Paediatric Hematology-Oncology and Bone Marrow Transplant Unit, Institute
of Child Health, Sir Ganga Ram Hospital, New Delhi.
Email: [email protected]
Introduction: The prevalence of bacteraemia in pediatric oncology ranges from 5-67%. Even
though most common etiology is Gram negative bacteria, Gram positive infections are also
present and can present in wide spectrum of clinical features and settings that we should be
aware of. Aims &Objectives: To describe spectrum of clinical manifestations due to S. aureus
sepsis in leukemia patients at our centre. Methods: Retrospective analysis of ALL patients
admitted at our centre from March- September 2012 was done. A total of 210 inpatient
episodes of ALL admitted in our ward and among them 40 had bacteremia. 3 cases with S.
aureus infection were detected. Results: Case 1: A 2-year-old boy had respiratory distress on
12th day of induction. Imaging showed bilateral pneumonia and splenic abscess. He was
given broad spectrum antibacterials and antifungals, despite which he had a parietal wall
abscess which grew methicillin resistant S.aureus (MRSA). Later he developed right
pneumothorax with empyema which also grew MRSA. He underwent VATS (video-assisted
thoracoscopic surgery) and is doing well. Case 2: 7-year-old boy with ALL (treatment
completed 2 years ago) presented with pain in the left hip after trivial trauma along with
fever. Imaging showed intracapsular collection. Both pus and blood culture grew methicillin
sensitive S.aureus (MSSA), he responded to antibiotics. Case 3: 20-year-old male known case
of ALL in maintenance phase had fever, swelling and discharge from the right testis. Incision
and drainage was done and pus culture showed MSSA, he responded to antibiotics.
Conclusion: This report highlights that ALL patients are susceptible to Gram positive
infections even though Gram negative infections are the commonest. There appears to be a
wide spectrum of S. aureus infection in ALL cases but literature in this regard is scarce
HO/47 (P) AUTOIMMUNE HEMOLYTIC ANEMIA IN INFANTS—A HARD NUT
TO CRACK
Sarita Verma P, Satya P Yadav, Vasant Chinnabhandar, Nivedita Dhingra, Anupam
Sachdeva
Division of Pediatric Hematology Oncology and Bone Marrow Transplant unit, Institute Of
Child Health, Sir Ganga Ram Hospital, New Delhi
Email: [email protected]
Objective – To highlight the varied spectrum autoimmune hemolytic anemia in infants.
Methods- 6 infants diagnosed as autoimmune hemolytic anemia based on the presence of
acquired hemolysis and confirmed by positive direct Coombs polyspecific test (DCT)
admitted from 2010 to 2012 were retrospectively reviewed. Clinical, laboratory, and outcome
data were obtained from patient records.
Results Age
Hb
Reti DC Cold Bone
findin Treatment
Foll Current
Sr At
TLC
c
T
agglu marrow g in Received
ow
status
no diagn Platelet coun
tinin
evaluati secon
Up
osis
count
t
on
dary
durat
%
work
ion
up
6
6.6
14.8 4+
Neg
Norm IV methyl pred 25
complete
1
mont 4500
Normal al
IV
Ig mont remission
hs
1.5 lac
dexamethasone, h
cyclophosphami
de,
azathiopurine
rituximab,
mycophenolate
mo
9
6.8
10.8 3+
Neg
Normal Norm IV
methyl 16
complete
2
mont 9900
al
prednisolone
mont remission
hs
ANC
and
oral h
neutropenia
396
prednisolone
persists
25,000
Is
in
regular
follow up
6
3.7
19
4+
Neg
Not
norm IV
methyl 22
Partial
3
mont 24,900
done
al
prednisolone
remission
hs
3.4 lac
and
oral mont Developed
prednisolone
h
thrombocyt
openia
Lost
to
follow up
2
4.1
6.4
4+
positi Not
Plasm Antimalarials
14
Complete
4
mont 2000
ve
done
odiu
Methyl
mont remission
hs
38,000
m
prednisolone
h
vivax Oral
prednisolone
7
4.3
13
4+
positi Not
Doub IV
methyl 11
Complete
5
mont 13,500
ve
done
le
prednisolone
mont remission
hs
2 lac
negati Oral
h
Kept
in
ve
prednisolone
regular
Tcells
follow up
–
6
4
mont
hs
3
16,300
1.6 lac
11
4+
positi
ve
normal
5.8%
Norm
al
IV Ig
3
Partial
IV
methyl mont remission
prednisolone
h
Oral
prednisolone
Conclusion: Ours is a small case series of infants with Autoimmune hemolytic anemia but the
difficult nature of the disease in infants is clearly evident. An exhaustive evaluation and
aggressive management is the key to sort it out.
HO/48 (P) CANCER IN CHILDREN WITH SYNDROMES: BAD LUCK AMPLIFIED
Vasant Ranganath Chinnabhandar, Satya Prakash Yadav, Nivedita Dhingra, Vikas Dua,
Anupam Sachdeva
Department of Pediatric Hematology-Oncology and Bone Marrow Transplant Unit, Sir
Ganga Ram Hospital, New Delhi, India
Email: [email protected]
Introduction: Oncotherapy in syndromic children is complicated by issues including
underlying syndromes restricting therapy tolerance, co-existing medical problems and socioeconomic issues. Aims and Objectives: To review data of syndromic oncology patients for
diagnosis, therapy and outcomes. Method: Retrospective analysis of cancer patients with
syndromes between January-2005 and January-2012 was performed. Result: Twenty-six
children, constituting 3.2% (n=825) of pediatric oncology patients identified. Of 11 (42.3%)
Down’s syndrome cases (commonest association) 7 (26.9%) had AML, two (7.7%) had
transient myeloproliferative disorder and one (3.8%) each had ALL and juvenile myelomonocytic leukemia. Three refused treatment, 3 died on-therapy and 5 were alive at latest
follow-up. Fifteen (57.7%) had rarer syndromes. Two (7.7%) had Fanconi anemia with AML,
both died. The Shwachmann-Diamond syndrome case with MDS was lost to follow-up. Of 2
(7.7%) cases with Greig syndrome and ALL, one is alive and disease-free 4 years from
diagnosis. One child with ataxia-telangiectasia and ALL died. Of 3 (11.5%) Wilm’s tumor
patients 2 had WAGR syndrome, another had hemi-hypertrophy, all completed treatment.
However one recurrence in the other kidney was noted 2 years post-therapy. Three (11.5%)
children from one family with identical mutations of von-Hippel Lindau gene developed
neuroblastoma, Burkitt's lymphoma and glioblastoma-multiforme respectively. Only the
Burkitt's lymphoma child is a long-term survivor. Among 2 (7.7%) hepatoblastoma patients
with maternal history of colonic adenocarcinoma, one was cured. One (3.8%) girl with
McCune-Albright syndrome and ovarian tumor and was successfully treated. At latest followup, 14 (53.8%) patients were alive and well. Conclusion: Outcome is compromised in
children with cancer if associated with a syndrome.
HO/49 (P) COMMON BILE DUCT ASCARIASIS AS A CAUSE OF
CHOLECYSTITIS IN A CHILD WITH ACUTE LYMHOBLASTIC LEUKEMIA IN
REMISSION
Sarita Verma P, Satya P Yadav, Mohit Kehar, Vasant Chinnabhandar, Nivedita Dhingra,
Anupam Sachdeva
Division of Pediatric Hematology Oncology and Bone Marrow Transplant unit, Institute Of
Child Health, Sir Ganga Ram Hospital, New Delhi
Email: [email protected]
Background: Worm infestations are common in the developing world. Biliary ascariasis
accounts for 10-19% of surgical complications of childhood ascariasis; common bile duct
(CBD) infestation is even rarer and has not been reported to cause of cholecystitis and severe
weight loss in pediatric acute lymphoblastic leukemia (ALL). Case: A 3-year old boy, with
precursor B-cell ALL, on interim maintenance as per modified UKALL-XI protocol
presented with multiple loose stools, vomiting, severe pain abdomen and fever. He was
dehydrated at presentation with right hypochondrium tenderness. Conservative management
with intravenous fluids and antibiotics was instituted. USG abdomen revealed a linear
echogenic focus in the gall bladder with dilated CBD. He improved transiently and was
discharged on oral antibiotics with ursodeoxycholic acid. Symptoms recurred after a few days
necessitating re-admission. ~35% weight loss over 3 weeks from base line was noted. Repeat
USG revealed persistent CBD dilatation with gall bladder edema. LFTs showed mild
hyperbilirubinemia and transaminitis. Pediatric gastroenterology opinion was taken; magnetic
resonance cholangio-pancreatography (MRCP) revealed a motile worm in CBD with
obstruction and dilatation of intrahepatic biliary ducts. Piperazine was given for 2 days, pain
and vomiting settled with return of appetite and gradual weight gain. He is presently doing
well and chemotherapy has been restarted with good tolerance. Message: In a developing
country like ours infections and infestations still remain common causes of complications
compromising treatment. Treatable specific causes of such manifestations should always be
kept in my mind as timely treatment can drastically improve the condition and avoid further
complications.
HO/50 (P) ACUTE ANTHRACYCLINE CARDIOTOXICITY IN A CHILD WITH
HODGKIN LYMPHOMA- A MIRACULOUS ESCAPE FROM DEATH
Anand Kumar K, Vasant Chinnabhandar, Nivedita Dhingra, Satya P Yadav, Anupam
Sachdeva
Division of Paediatric Hematology-Oncology and Bone Marrow Transplant Unit, Institute of
Child Health, Sir Ganga Ram Hospital, New Delhi.
Email: [email protected]
Overall incidence of clinical heart failure due to anthracyclines is 16% and most of it is late
onset seen in the survivors of cancer but early onset cardiotoxicity can occur (<1%).
Screening for known risk factors and serial cardiac monitoring is vital in prevention of this
event. Even though the risk is highest at cumulative dose of >300mg/m2, no dose is safe.
Case report of acute anthracycline toxicity admitted at our centre in June 2012. 7 year old girl
with Hodgkin lymphoma came for cycle 2A of ABVD chemotherapy. She developed signs
and symptoms of congestive heart failure with cardiogenic shock one day after she received
the treatment. She had multiple episodes of cardiac arrest and was revived after aggressive
and prolonged CPR. She had persistent lactic acidosis for >24 hours, required support of
multiple inotropes, vasopressors and mechanical ventilation. Sepsis workup and viral PCR
for possible myocarditis was negative. Her cardiac evaluation showed she had non-specific
ECG changes, 2D-echo showed ejection fraction <20%. She developed multiorgan hypoxic
ischemic insult. Appropriate support with fluid, electrolytes and blood components was
provided. After 2 weeks on mechanical ventilation her organ functions improved and she was
weaned off with intact neurological outcome and she is doing well. Conclusion: Acute
anthracycline cardiotoxicity even though rare can occur and the cardiac insult is temporary,
prior cardiac evaluation is a must before treatment. Last but not least “HOPE KEEPS LIFE
ALIVE” it was due to some relentless and persistent effort by the treating team that this child
survived amidst the worst case scenario.
HO/51 (P) A NOVEL RAG-1 MUTATION IN AN INFANT WITH SEVERE
COMBINED IMMUNODEFICIENCY AND CENTRAL NERVOUS SYSTEM
INVOLVEMENT
Nivedita Dhingra, Satya Prakash Yadav, Capucine Picard, Vasant Chinnabhandar, Veronique
Dinand, Anupam Sachdeva
Pediatric Hematology Oncology and Bone Marrow Transplant Unit, Sir Ganga Ram Hospital,
New Delhi, India
Email: [email protected]
Introduction: Known mutations in recombinase-activating genes (RAG-1and 2) account for
10% cases of severe combined immunodeficiency (SCID). In RAG-1 deficient SCID central
nervous system (CNS) pathology is usually infectious and primary CNS involvement has not
been described. Aim: To describe an unusual case of SCID with CNS manifestations in
which a novel RAG-1 mutation was identified. Method: Patient –A 15-month old boy, 2nd
born of a consanguineous marriage, presented with failure to thrive and recurrent infections
and was diagnosed with T-B-NK+ SCID on immunological evaluation. He developed right
complex partial seizures with ipsilateral hemiparesis and became comatose. MRI brain
revealed an inflammatory lesion in the left thalamus which later progressed to diffuse
meningo-encephalitis on serial imaging. Evaluation for an infectious etiology was negative
for all common viral, fungal and bacterial pathogens (including CMV, EBV, HSV,
adenovirus, JC virus, toxoplasma and JE) by culture, serology and PCR as available. Genetic
work-up revealed a novel homozygous deleterious mutation in the RAG-1 gene(c:2881T>C;
p:I794T) in the child for which both parents were heterozygous. No mutation found in RAG2
and artemis despite sequencing. Empirical therapy with multiple antibacterial, antiviral,
antifungal, antiprotozoal agents was administered. He underwent a haplo-identical bone
marrow transplant (mother was donor) without conditioning. He died on day +35 posttransplant with rejection of donor cells and no improvement in the neurological status.
Conclusion: A new mutation in RAG-1 gene was identified. Failure to identify an infectious
agent led to a possibility of primary CNS involvement which has not been described so far in
this variant of SCID
HO/52 (O) DETERMINANTS OF NUTRITIONAL ANEMIA IN CHILDREN LESS
THAN FIVE YEARS AGE
Sandip Ray, Jagdish Chandra, Jayashree Bhattacharjee, Sunita Sharma, Anuja Agarwala
Department of Pediatrics, Kalawati Saran Children’s Hospital, New Delhi, India.
Email: [email protected]
Abstract: Introduction : According to WHO, highest prevalence of anemia is found in preschool aged children, and a large proportion of them live in India. Data on factors associated
with anemia in this vulnerable age group in India are limited. Aims & Objectives : The
objective of this study was to determine demographic, socio-economic & nutritional factors
for nutritional anemia in children <5 yrs. Material And Methods : We conducted a crosssectional study of children aged 6 to 59 months. Hemoglobin ( child & mother ), ferritin,
folate, vitamin B12 levels were determined. Anthropometric measurements, nutritional
intake, family income and other demographic data were recorded. Results : Majority of cases
(75.0%) were in 6months – 2 years age group. 139(69.5%) subjects had iron deficiency,
86(43.0%) had Vitamin B12 deficiency and 28(14.0%) had folic acid deficiency. On
univariate analysis, higher age, high birth order, lower income, low education of father,
higher number of family members, symptoms of anemia in mother and intake of iron were
significantly associated with severity of anemia. However, on multivariate regression the
factors found to be associated were : increasing birth order, low iron intake & symptoms of
anemia in mother. Conclusions: Low iron intake, increasing birth order and symptoms of
anemia in mother were significant factors associated with nutritional anemia in children less
than 5 years age. Thus, strategies for minimizing childhood anemia must include optimized
iron intake through increased dietary iron intake or iron supplements but should
simultaneously address maternal anemia and family planning measures.