IMMUNE THROMBOCYTOPENIC

PURPURA

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 INTRODUCTION

• Platelets are non nucleated cellular fragments

• Produced from megakaryocytes in the bone marrow
• Megakaryocytes are large polypoid cells
• Mature megakaryocyte - budding of cytoplasm
occurs & large number of platelets are liberated into
circulation
• Thrombopoeitin- primary growth factor controls
platelet production

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 INTRODUCTION

• Normal platelet count

Newborn 84,000 - 4,78,000 cells/ cu mm
After 1 week - adults - 1,50,000- 4,00,000 cells/mm3
• Average life span of platelets- 7- 10 days
• Decrease in platelet count - Thrombocytopenia
• Increase in platelet count - Thrombocytosis

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 THROMBOCYTOPENIA

• Platelet count - < 1,50,000 cells/cu mm

• Causes
• Increased platelet sequestration
Hypersplenism, Hypothermia, Burns
• Increased platelet destruction
• Immune, Non immune
3. Decreased platelet production

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 CUT - OFF VALUES

• Neonates - 20,000 - 50,000 cells/mm3

[high risk for ICH]
• Children - 10,000- 20,000 cells/ mm3
• Spontaneous bleeding do not occur until counts are
< 20,000 cells/mm3

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 IMMUNE THROMBOCYTOPENIC PURPURA

• Commonest bleeding disorder manifesting in

children.
• Usual age of presentation - between 1-7 years.
• Acute ITP- lasts less than 6 months.
• Chronic ITP- lasts more than 6 months.
• The majority of children [60-75%] are likely to have
acute ITP and that resolves within 2-4 months of
diagnosis, regardless of therapy.

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 ITP - PATHOGENESIS

• Secondary to antibodies directed against the platelet

glycoprotein II b/ III a complex
• These platelets are trapped in the spleen, where
they are removed by macrophages
• Mechanism of production of antibodies is not known
• Hall mark is increased number of megakaryocytes in
the bone marrow
• Th1 dominant pro- inflammatory cytokine state is
also described recently

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 ACUTE ITP

• Benign, self limited

• Young children [<10 years ]
• Often Viral infection or Vaccination precedes
• Resolves within weeks or a few months of original
presentation.

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 CHRONIC ITP

• Persistence of Thrombocytopenia [ <150000/mm3 ]

for longer than 6 months after initial presentation
• > 10 years
• Insidious onset
• Female gender.

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 ITP -CLINICAL FEATURES

• Abrupt onset of bruising & bleeding in an otherwise

healthy child
• H/O preceding viral illness or vaccination +/-
• Seasonal clustering of cases and more frequent
during change of seasons
• Petechiae, purpura & ecchymosis
• Epistaxis & oral mucosal bleeds occur in < 1/3rd
• Hematuria, hematochezia, melena in < 10%
• Menorrhagia
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 ITP - CLINICAL FEATURES ( CONT..)

• Palpable spleen - < 10% cases

• Symptoms & signs depend on the platelet count
• Bleeding is mild unless the platelet count falls
<20,000/cu mm
• Petechiae & ecchymosis can occur following mild
trauma with a platelet count of 20000-50000/mm3
• No dysmorphic features/bony anomalies/
hyperpigmentation
• Suspect malignancy when there is malaise, bone pain,
organomegaly or lymphadenopathy

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 CLASSIFICATION - BASED ON SYMPTOMS
1. No symptoms
2. Mild symptoms - bruising , petechiae, occasional
minor epistaxis & very little interference with daily
living
3. Moderate symptoms - more severe skin & mucosal
bleeds, more troublesome epistaxis & menorrhagia
4. Severe symptoms - bleeding episodes -
menorrhagia, epistaxis, melena- requiring
transfusion or hospitalization, symptoms interfering
seriously with the quality of life

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 INVESTIGATIONS
• Complete blood count:-
• Low platelet count, Hb% and WBC counts are normal
• Peripheral smear evaluation
• To see all cell lines, to estimate the platelet count & to
exclude spurious thrombocytopenia, to examine for
abnormal cells [ blasts] or malarial parasites
• Circulatory platelets are larger in size, indicating increased
production
• Liver function tests & Lactate dehydrogenase levels
• To rule out Hepatitis, Malignancy & Hemolysis

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 INVESTIGATIONS

• Evaluate all cases with fever for infection

• Screening tests for disseminated intravascular
coagulopathy when sepsis is suspected
• Bone marrow examination- Aspiration/Biopsy
indicated in
• Abnormal WBC count or differential
• Unexplained anemia
• History & physical examination suggestive of bone
marrow failure
• Failure of initial therapy
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 ADDITIONAL TESTS [CHRONIC CASES]

• Screening tests for Immunodeficiency

• Anti nuclear antibodies
• Direct Coomb’s test
• Anti phospholipid antibody assay
• Thyroid function tests
• Blood counts and peripheral smear for parents
• Serology testing for platelet antibody is seldom
useful.

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 MANAGEMENT

• Child’s activity should be limited

• Aspirin containing medication should be avoided
• Detailed education & counseling of the family and
patient
• Careful follow up
• Observation only
Platelet count >20,000/mm3 & only minor purpura
• Platelet transfusion in ITP is contra indicated unless
life-threatening bleeding is present
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 FIRST LINE MEDICAL THERAPIES

• Corticosteroids
• Intra venous Immunoglobulin
• Anti D

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 CORTICOSTEROIDS
• Inhibition of both phagocytosis & Antibody
production
• Improved platelet production and micro vascular
endothelial stability
• Dose
Prednisolone - 1-4 mg/kg/day x 2-3 weeks &
tapered Dexamethasone - 20mg/mt2 over 4 days
every 3 weeks for 4-6 courses
Alternate- mega pulse dose therapy
Methyl Prednisolone 30mg/kg/day IV / oral x 3days

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 INTRA VENOUS IMMUNOGLOBULIN

• Clearance of antibody coated blood cells from the

circulation by inhibiting the phagocytic activity of
cells of reticulo-endothelial system
• Fc receptor blockade
• Dose - 2gm/kg divided over 2-5 days
• Expensive but cost effective
• Side effects:- flu like symptoms, aseptic meningitis,
anaphylaxis, hemolytic anemia, HCV transmission
• Duration of response is brief [2-4 weeks]

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 ANTI - D

• Plasma derived Immunoglobulin prepared from

donors selected for a high titer of anti Rh antibody
• Elicits an increase in platelet counts along with mild
to moderate anemia
• Can only be used in Rh positive patients
• Dose - 50-75 micro gm./kg as IV infusion
• Therapeutic effect lasts for 1-5 weeks
• Side effects:- headache, nausea, chills, dizziness,
fever, hemolysis

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 MANAGEMENT OF CHRONIC ITP

• Primary goal - prevent bleeding episodes & not to

cure the disease
• 1/3 of these children will go into remission in months
or years later
• Observation alone is an approach for many patients,
especially those with minimal symptoms

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 SPLENECTOMY

Indications for Splenectomy

•Children with ITP for more than 1 year
•Symptoms of bleeding and counts
• < 10000/mm3 [ 3-12 yrs. of age]
• 10000- 30000/mm3 [8- 12 yrs. of age]

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 MANAGEMENT OF C/C ITP

• First line Medical therapy:-

Intermittent Steroids, IV Ig, Anti D
• Second line therapy - for 10- 15% of children who
fail to respond to first line drugs / splenectomy
• Danazole - 300- 400 mg/m2/day orally for 2 months
• Vinca Alkaloids - Vincristine 1.5 mg/m2 IV weekly x 1
month
• Azathioprine - 50-200 mg/m2/day orally for 4- 6
months
• Cyclosporine - 5mg/kg/day in divided dose
• Cyclophosphamide & Interferon may be used
• Plasmapheresis may be done
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 LIFE THREATENING HEAMORRHAGE

• Intra venous Immunoglobulin- 1gm/kg

• Anti D - 50- 75 microgram/kg as IV Infusion
• High dose Corticosteroid - IV Methyl Prednisolone
30mg/kg
• Platelet Transfusion
• A combination of above can be tried

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 PROGNOSIS

• Excellent chance of recovery, irrespective of therapy

• Platelet count returns to normal in 4-8 weeks in ½ of
the patients and in 2/3 of children by 3 months after
diagnosis
• 5% have recurrent ITP, characterized by intermittent
episodes of thrombocytopenia followed by lengthy
periods of remission

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 THANK YOU

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