Download Case Conference Presentation 10/19/2011

Case Conference Presentation
Tania Kourtidou, MD
PGY-1
10/19/2011
Case Description
14yo girl with c/o b/l lower leg rash x1d
Case Description
14yo girl with c/o b/l lower leg rash x1d
•
Red spots, nontender, non-pruritic
•
Applied Hydrocortisone cream ->no change
•
Afebrile, no URI, GI, GU, musculoskeletal, visual disturbance
•
On the 4th day of menstrual cycle
•
1st episode, no Hx of trauma, insect bite, pets, recent travel,
change in soaps, -sick contacts
Case description
 PMHx: Tested for Marfan Syndrome – Negative
 FH: Asthma, DM, HTN, Seizure disorder
 Immunization status: Unknown
 SHx: Denied smoking, sexual activity, illicit drug use, suicidal
thoughts, both parents at home, attended high school.
Case Description
 VS: T 98.2F, HR 98, BP 108/72, RR 20, SpO2 100%
 Physical exam:
Skin: Flat, hemorrhagic, non-blanching, pinpoint, non tender,
located only in lower extremities, ant>post
Rest of PE: wnl
Petechial - Porpuric rash
Petechea <3mm
Porpura: 3-10mm
Glass test
Petechial - Porpuric rash
Differential diagnosis
Meningococcal
infection
Viral infections
*Enterovirus
Leukemia
Malignancy
HSP
ITP
D.I.C.
Pressure
Vomiting
Trauma
*Simple porpura indicates a qualitative or quantitative PLTs disorder.!
Case Description
Lab tests:
1. CBC, RET count, Peripheral smear
2. PT, aPTT, INR
3. ERS, CRP
4. CMP
5. Blood Cx
6. *UA could not be evaluated for blood
Case Description
Lab results:
40.5
6.2
13.7
11.6
1.1
118
140
106
10
4.6
29
0.6
7.5
13
0.9
4.7
21
9.6
94
94
0.9
25.7
ESR: 2
Peripheral smear:
(no schistocytes, blasts)
CRP, Blood Cx: Negative
Case Description
THROMBOCYTOPENIA
Destruction
Production
Congenital
Aplastic anemia
Infections
(HIV,HCV,H.pylori)
Paroxysmal nocturnal
hemoglobinuria
Von Willebrand’s IIB
Drugs
Immune
Non-immune
ITP
DIC
Alloimune (neonate)
TTP/HUS
Infections
Kassabach-Merrit
Drugs
syndrome
SLE, Antiphospholipid Hypersplenism
syndrome
Fleisher GA, LudwigS, et al., eds. Textbook of pediatric emergency medicine. 3d ed. Baltimore: Williams & Wilkins, 1993:430–8.
Case Description
Based on:
 clinical presentation
 PLTs
 Normal CBC and peripheral smear
Pt d/c with the diagnosis of ITP
No indication for treatment
Recommended to f/u with hematology clinic
Idiopathic Thrombopenic Porpura
 Porpura ~>
Πορφύρα (porphyra)
Colouring substance produced with the treatment of
shell Haustellum brandaris .
Gives indelible deep red colour.
It was particularly precious because of its difficult
production and the rarity of shells.
Therefore the use of clothing dyed with pupura
represented a sign of wealth and power.
Emperor’s cloak in the Empire of the Byzantium was
always colored with porpura.
Justinian I
Idiopathic Thrombopenic Porpura
Outline
1. Introduction
2. Pathogenesis
3. Diagnosis
4. Clinical manifestations
5. Therapeutic principals
6. Latest treatment options
Idiopathic Thrombopenic Porpura
Isolated persistent thrombocytopenia: PLTs<100x109/L
 Normal CBC
 Normal peripheral smear
Idiopathic Thrombopenic Porpura
 The most common cause of thrombocytopenia in children
 Prevalence: 4.0-5.3/100.000, 3500 new cases per year
 85%
Acute and self-limited
 Between 2 and 10 years (peak age: 5y)
>10y girls
Chronic ITP
Insidious presentation
 Equal gender/ethnic distribution
>6m
Idiopathic Thrombopenic Porpura
The etiology is still unknown.
Idiopathic Thrombopenic Porpura
Pathogenesis
*1-4week after exposure to common viral infection or
immunization (varicella, MMR).
Theories
Antibody crossreactivity
H. pylori
bacterial
lipopolysaccharides
Idiopathic Thrombopenic Porpura
Pathogenesis
Antibodies against viruses may x-react to
antigensimmune
complexes
on
the
surfaceremoval by reticuloendothelial system
PLT
PLT
Some strains of H. pylori may induce PLT aggregation
Bacterial products (ex.LPS) once adhered to PLTs, may
induce increased phagocytosis or “clearance” of PLTs
Pathophysiology
*B cells produce IgG
autoantibodies against GP
IIb/IIIa and Ib/IX.
Idiopathic Thrombopenic Porpura
Clinical manifestations
• Sudden onset
• Healthy child
• Mucocutaneous bleeding:
epistaxis, gum bleeding, menorrhagia
GI or CNS
<1%
• 50% Minimal splenomegaly
Idiopathic Thrombopenic Porpura
Diagnosis (of exclusion)!!!
PLTs<100x109 /L
Hb
Normal aPTT and PT
Prolonged BT
Peripheral smear:
Megathrombocytes
Idiopathic Thrombopenic Porpura
Diagnosis
 Bone Marrow aspiration
Normal or Increased
number of
megakaryocytes
 Anti-PLT antibody studies
 Sensitivity
and specificity
 +ANA (adolescents)
 EBV, CMV, Mycoplasma, H.pylori (cITP)
Idiopathic Thrombopenic Porpura
These laboratory tests are NOT recommended by
the ASH practice guidelines to patients with the
typical ITP presentation.
(American
Society of Hematology, 2011)
Diagnosis of ITP should be based on :
1. Infection history
2. Clinical features
3. Physical exam
4. Lab tests: CBC and peripheral blood smear
Idiopathic Thrombopenic Porpura
Treatment
Most of the cases can be managed at home
“Most patients and their parents can live quite comfortably with
petechiae and low platelets awaiting spontaneous remission
providing their physicians can.”
Dickerhoff 1994, Thrombocytopenia in childhood.
Idiopathic Thrombopenic Porpura
Treatment guidelines
If skin manifestations only
Observation regardless of the PLTcount
Close monitor of CBC once a week
Once PLT begin to increase, it takes 23 weeks to normalize.
Hospitalize if
Severe, life threatening bleeding
regardless of the PLT count
Idiopathic Thrombopenic Porpura
Treatment guidelines
Consider treatment if PLTs<30x109/L
The goal is to raise PLT count hemostatically safe, not to cure
Single dose of IVIG
(0.8-1g/kg xor
2d or 400mg/kg/d x 5d)
1st line treatment:
Short course of corticosteroids
(Methylprednisolone 20-30mg/kg
x3d or Prednisone 1-2.g/kg/d x14d)
Idiopathic Thrombopenic Porpura
Treatment guidelines
IVIG:
 Blocks Fc receptor on phagocytes  PLT destruction
 Rapid elevation of PLT count >20.000 within 48h  Preferred to
corticosteroids in severe disease
 Expensive, long effusion time (6-8h), allergic reactions, aseptic meningitis
Idiopathic Thrombopenic Porpura
Treatment guidelines
Corticosteroids:
 Reduce capillary fragility, inhibit PLT destruction and antibody production
 No evidence supporting long course vs. brief course
 Cheap and convenient but side effects of long term use
Idiopathic Thrombopenic Porpura
Treatment guidelines
IV Anti-D therapy:
 1st line for Rh+ with functional spleen
 Induces mild hemolytic anemia RBC-antibody complexes saturate
the macrophage Fc receptors  Increased survival of antibodycoated PLTsslow rise of PLTs
 Less allergic reactions than IVIG, no aseptic meningitis
 Hemolysis Transient  Hb
Idiopathic Thrombopenic Porpura
Treatment guidelines
Splenectomy:
Reserved for children >4 years of age with persistent
symptoms (bleeding) lasted longer than 1 year and lack
of response to therapy and/or who have a need for
improved quality of life.
Idiopathic Thrombopenic Porpura
Treatment guidelines
Transfused PLTs
No role in the routine
management of ITP
*Rapidly removed from the circulation  only used in emergencies to control
bleeding (PLT<3x109/L)
Idiopathic Thrombopenic Porpura
Life threatening hemorrhage:
1.PLT infusions (10ml/kg expect to  PLT by 50.000/L)
2. IV Methyprednisolone 30mg/kg (max 1g) over 20min, repeat daily
up to x3
2.IVIG (1g/kg over 4-6h, repeat daily up to x5)
3.Emergent splenectomy
4.Plasmapheresis, RBCs transfusion, antifibrinolytics
Idiopathic Thrombopenic Porpura
Special Considerations
TPO Mimetics and
Receptor Agonists
(Romiplostim,
Eltrompobag)
No published data to guide the use of these agents
in children
High-Dose
Dexamethasone
Adolescents with significant ongoing bleeding and/or
need for improved quality of life despite conventional
treatment.
Rituximab
A-Interferon
Altenative to splenectomy or in those who have
failed splenectomy.
Immunosuppression
(AZA,CTX,VCR)
Multiple agents have been reported; however
insufficient data for specific recommendations.
Idiopathic Thrombopenic Porpura
Follow up:
 Spontaneous recovery:
 10-20% chronic ITP
-60% by 3months
-80% by 6months
-90% by 1year
adolescent girls
 monitor platelet count and clinical status daily to weekly,
depending on the severity and treatment
 Once platelet count has normalized, recurrence is rare and followup platelet counts are unnecessary
Idiopathic Thrombopenic Porpura
 Parent education.
 Avoidance of contact sports, wearing protective
headgear, lining the crib with protective padding
 MMR should be given regardless PMHx of ITP
 Discontinue medications that suppress platelet
production
 There should be a low threshold for prompt evaluation
of child that has sustained blunt trauma with ITP.
Idiopathic Thrombopenic Porpura
 ITP is often an acute and self-limiting disease in
children
 Most of the times no treatment is required
 Goal = prevention of complications
 Therapy needs to be tailored to the individual patient
 Parent and patient education is very important
References:

2011 Clinical Practice Guideline on the Evaluation and Management of Immune
Thrombocytopenia (ITP), The American Society of Hematology

Idiopathic Thrombocytopenic Purpura in Children: Diagnosis and ManagementP. D.
McClure Pediatrics 1975;55;68

Evaluating the Child with Purpura, Leung et al., Am Fam Physician 2001;64:419-28

Childhood Immune Thrombocytopenic Purpura: Diagnosis and Management,
Blanchette V., Bolton-Maggs P., DM, Pediatr Clin N Am 55 (2008) 393–420

www.uptodate.com

www.aap.org

www.hematology.org

www.pdsa.org
 A 9-year-old boy presents to your office with purple spots on his
legs and mild swelling of his scrotum of 1 day's duration. He has
had no vomiting, diarrhea, or constipation. He is afebrile, alert, and
active. On palpation, he reports mild abdominal discomfort. He has
no edema of the lower extremities or presacral area. His weight is
1 kg more than his weight at his health supervision visit 6 months
ago.
Of the following, the MOST likely abnormal laboratory
finding to expect for this boy is:
A. anemia
B. hypoalbuminemia
C. microscopic hematuria
D. prolonged partial thromboplastin time
E. thrombocytopenia
A. anemia
B. hypoalbuminemia
C. microscopic hematuria
D. prolonged partial thromboplastin time
E. thrombocytopenia