Download 07-02-51

Extern Conference
Supervirsor
Doctor Bunchoo Pongtanakul
Doctor Nithiwat Vatanavicharn
An 11-year-old girl
Chief complaint
Neck mass at Lt side 6 months PTA
Present History
6 mo PTA
The patient’s mother noticed that the patient’s left
neck was enlarged. Later her mother decided to take
her to the hospital. The clinician told that she had
enlarged lymph node and prescribed her oral
antibiotics for 2 weeks.
Present History
2 weeks later
Her clinical symptoms did not improve and the lymph
node biopsy was done. But the pathological report
suggested an inadequate tissue. So the clinician
decided to continue oral antibiotics.
Present History
2 mo PTA
Her lymph node was progressively enlarged, and her
mother noticed that the right side was enlarged too.
At the hospital, the physical examination was
performed and reviewed that preauricular,
submandibular and anterior cervical node
enlargement both sides.
Present History
2 mo PTA
The lymph node biopsy was done again, and the
pathological report suggested malignant lymphoma.
The clinician referred the patient to Siriraj Hospital
for further management.
Present History
1 mo PTA
She complained about bloating, loss of appetite and
weight loss1 kg in 1 mo.
Present History
She had no history of fever, night sweating,
bleeding, epistaxis, pale, dyspnea or chronic
cough.
No history of dysphagia, oral ulcer, oral thrush or
hearing loss before.
No palpable mass at the other sites.
No history of contact TB.
Past History
No significant medical history.
No previous surgery.
No history of head and neck trauma.
Family History
No history of malignancy in the family.
Allergic History
No history of drugs, food or chemical allergy
before.
Physical Examination
V/S : T 37.2 ºc, RR 14/min, PR 84/min, BP
104/65 mmHg
GA : Thai 11-year-old girl, alert and active,
sthenic built, not pale, no jaundice, no edema,
no dyspnea, no tachypnea
Skin : no rash, no petechiae, no ecchymosis
Physical Examination
HEENT :
Head : normocephalic, atraumatic
Eye : WNL
Ear : WNL
Nose : normal mucosa, no visible mass
Throat : pharynx and tonsils not injected
Physical Examination
RS : normal breath sounds, no adventitious
sounds
CVS : normal S1 and S2, no murmur
Abdomen : mild distend, soft, no tenderness,
liver just palpable, liver span 7 cm, spleen 3 FB
below LCM, active bowel sounds
Physical Examination
GU : WNL
NS : E4V5M6, pupil 3 mm BRTL, full EOM, no
visual field defect, no facial palsy, gag reflex +ve,
Rinne’s BC>AC both, Weber’s no lateralization,
motor power grade V all, sensory intact, stiff
neck and Kernig’s sign -ve
Physical Examination
Lymph Node :
Multiple cervical lymphadenopathy vary in size 0.5-2
cm in diameter
Lt epitrocheal node 1.5 cm in diameter
Both inguinal node 0.5-1.5 cm in diameter
No tenderness, rubbery in consistency, smooth
surface, movable, no signs of inflammation
Initial Investigation
CBC
Peripheral blood smear
CXR
CBC
18/1/2008
CBC
30/1/2008
Peripheral Blood Smear 30/1/08
Normochromic normocytic RBC
Platelet about 15-20 cell/OF. No platelet
aggregation.
WBC : L 60%, N 39%, M 1%. No blast.
Electrolyte
18/1/2008
CXR 18/1/08
Intact bony structure
Normal soft tissue
Minimal widening of upper mediastinum
No pulmonary infiltration.
Cardio-thoracic ratio 0.46
Problem List
Generalized lymphadenopathy at cervical,
epitrocheal and inguinal region for 6 mo
Bloating, loss of appetite and weight loss for 1
mo
Splenomegaly
Pancytopenia with lymphocytosis
Lymphadenopathy
Lymphadenopathy
The body has 600 lymph nodes
Only in the submandibular, axillary or inguinal
regions may normally be palpable
Lymphadenopathy refers to nodes that are
abnormal in either size, consistency or number
Lymphadenopathy
Size
Lymphoid mass increases steadily after birth
until age 8-12 yrs and undergoes progressive
atrophy during puberty
Newborns usually have small adenopathy (<0.5 cm)
In young children :
• Anterior cervical nodes as large as 1.5 cm
• Axillary nodes as large as 1 cm
• Inguinal nodes as large as 1.5 cm
Should be considered abnormal if the epitrochlear or
supraclavicular nodes larger than 0.5 cm.
Lymphadenopathy
Localized
If only one area is involved
Generalized
If lymph nodes are enlarged in two or more
noncontiguous areas
Axillary
Epitrochlear
Cervical
Inguinal
Approach to Generalized
Lymphadenopathy
Generalized Lymphadenopathy
239 children underwent peripheral lymph node biopsies
for evaluation of lymphadenopathy. The etiology were
noted
Reactive hyperplasia
Granulomatous disease
Neoplastic disease
Chronic dermatopathic or bacterial infection
52%
32%
13%
3%
From Knight PJ ; Mulne AF ; Vassy LE : Pediatrics 1982 Apr ; 69(4) : 391-6
Historical Clues
Age and duration
The vast majority of cases of lymphadenopathy in
children is infectious or benign in etiology.
Lymphadenopathy that lasts ≤ 2 weeks or ≥ 1 year
with no progressively increasing in size has a very
low likelihood of being neoplasm.
Historical Clues
Exposure
Exposure to animals
Travel-related exposures and immunization status
Environmental exposures such as tobacco, alcohol
Ultraviolet radiation
Patients with AIDS
Historical Clues
ASSOCIATED SYMPTOMS
Constitutional symptoms such as fatigue, malaise,
and fever, significant fever, night sweats and
unexplained weight loss
Symptoms such as arthralgias, muscle weakness, or
unusual rash may indicate the possibility of
autoimmune diseases
Generalized Lymphadenopathy
Infection
Malignancy
Other
Generalized Lymphadenopathy
Infection
• Infectious Mononucleosis
• HIV
• CMV
• Varicella
• Adenovirus
• Roseola Infantum
• Salmonella typhi
• Syphilis
• Plague
• Tuberculosis
Generalized lymphadenopathy
Malignancy
• ALL
• AML
• Lymphoma
• Langerhans cell histiocytosis
• EBV associated lymphoproliferative disease
Generalized lymphadenopathy
Other
• Drugs
• Autoimmune disease
eg. JRA , SLE
Diffential Diagnosis
Hematologic malignancy
Lymphoma
Acute leukemia
Chronic infection
Tuberculosis
HIV infection
Hematologic Malignancy
Pro
Generalized
lymphadenopathy
Splenomegaly
No response to ATB
Abnormal CBC :
pancytopenia with
lymphocytosis
Cons
No sign of BM failure
(Acute leukemia)
Chronic Infection
Pro
Generalized
lymphadenopathy
No response to ATB
Cons
No chronic cough
No Hx of contact TB
Investigation
Lymph node biopsy
Lymph Node Biopsy
Left cervical lymph node biopsy:
Precursor T lymphoblastic lymphoma
A complete hematologic work-up is highly
recommended to exclude acute lymphoblastic
leukemia of T-cell phenotype (T-ALL).
BM Aspiration
BM Aspiration
Diluted BM, mild hypocellularity, normal
megakaryocyte, decreased erythroid and
myeloid series, increased lympoid series,
lymphoblast 25-30%
Final Diagnosis
Acute Lymphoblastic Leukemia
( T Cell )
Introduction
Acute leukemia is the most common cancer in
children
ALL > AML ~ 5
Peak incidence 2-5 yrs
Signs and Symptoms
Musculoskeletal : bone pain
Lymphadenopathy ~50%
Headache ~5%
Testicular enlargement
Mediastinal mass
Peripheral blood abnormalities
Anemia
Thombocytopenia
Lymphoblast on peripheral blood
Diagnosis
The diagnosis and classification of leukemia are based
upon specialized tests that are performed on cells
derived from a bone marrow aspiration or tissue biopsy
specimens
ALL is the preferred term when the bone marrow
contains > 25 % lymphoblasts, whereas lymphoma is
the preferred term when the process is confined to a
mass lesion with minimal or no blood and marrow
involvement
Risk assignment and suggested
therapies
Risk
Group
Features
%
Lesser
Hyperdiploid or trisomies 4, 10, 17
20
t(12,21)
20
Standard WBC <50,000/microL
Conventional antimetabolite-based
therapy
15
Intensified antimetabolite
therapy
T-cell phenotype
15
Age >10 years
15
Intensive multi-agent
therapy
WBC>50,000/microL, t(1;19)
6
Age 1 to 9.9 years
High
Recommended
Therapy
Very high t(9;22)
3
t(4;11); age <1 year
4
Induction failures and slow
responders
2
Consider allogeneic
hematopoietic cell
transplantation in first
remission
Adverse Effects
Tumor lysis syndrome
Thrombosis
Bleeding
Infection
Tumour Lysis Syndrome
The term applied a group of metabolic
complication that usually occur after the
treatment of neoplastic disorder
Eg. ALL, Burkitt’s lymphoma, T cell leukemia
lymphoma
Findings
Hyperphosphatemia
Hypocalcemia
Hyperuricemia
Hyperkalemia
Management
Prevention of tumour lysis syndrome
Adequate hydration : at least 2 times of MT, adjust q
2-3 hr, keep urine sp.gr. < 1.010
Urine alkalinization : add NaHCO3 30-100 mEq/L ,
keep urine pH 6.5-7.5
Allopurinol 10 mg/kg/day q 8 hr
Management (2)
Treatment : correct metabolic disturbance
Hyperkalemia : NaHCO3 , insulin, glucose, 10%
calcium gluconate, Kayexalate
Hyperphosphatemia : Ca X PO4 > 60 , Give
Aluminium hydroxide 150 mg/kg/day
Hypercalcemia : 10% calcium gluconate 0.5-1 ml/kg
or calcium chloride 10 mg/kg, EKG monitoring
Hemodialysis