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Key Features

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Essentials of Diagnosis
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  • Otherwise healthy child

  • Decreased platelet count

  • Petechiae, ecchymoses

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General Considerations
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  • Most common bleeding disorder of childhood

  • Occurs most frequently in children aged 2–5 years

  • Often follows infection with viruses, such as

    • Rubella

    • Varicella

    • Measles

    • Parvovirus

    • Influenza

    • Epstein-Barr virus (EBV)

    • Acute and chronic HIV

  • Most patients recover spontaneously within a few months

  • Chronic ITP (> 12 months' duration) occurs in 10–20% of affected patients

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Clinical Findings

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  • Sudden onset, with appearance of multiple petechiae and ecchymoses

  • Epistaxis is common at presentation

  • Rarely, concurrent infection with EBV or CMV may cause hepatosplenomegaly or lymphadenopathy, simulating acute leukemia

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Diagnosis

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  • Platelet count is markedly reduced (usually < 50,000/μL and often < 10,000/μL)

  • Platelets are frequently large on peripheral blood smear, suggesting accelerated production of new platelets

  • Platelet-associated IgG or IgM, or both, may be demonstrated on the patient's platelets or in the serum

  • Prothrombin time and activated partial thromboplastin time are normal

  • White blood count and differential are normal

  • Hemoglobin concentration is preserved unless hemorrhage has been significant

  • Number of megakaryocytes is increased

  • Erythroid and myeloid cellularity is normal

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Treatment

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General Measures
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  • Observation is recommended for most children in the absence of bleeding regardless of platelet count

  • Food and medications (eg, aspirin and nonsteroidal anti-inflammatory drugs) that compromise platelet function should be avoided

  • Bleeding precautions (eg, restriction from physical contact activities and use of helmets) should be observed

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Medications
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  • Corticosteroids

    • Short course may benefit patients with clinically significant but non–life-threatening bleeding and those with a platelet count of less than 10,000/μL

    • Dosing

      • Initial dose of prednisone 3–5 mg/kg/d for 3–7 days may lead to faster count recovery

      • Additional prednisone not given unless significant bleeding recurs, at which time smallest dose that achieves resolution of bleeding episode is administered (usually 2.5–5 mg twice daily)

    • Long-term use should be avoided because of toxicity

  • Intravenous immunoglobulin (IVIg)

    • Treatment of choice for severe, acute bleeding

    • May be used as an alternative or adjunct to corticosteroid treatment

    • A single dose of 0.8–1 g/kg has been recommended

    • Platelets may be given simultaneously during life-threatening hemorrhage but are rapidly destroyed

  • Anti-Rh(D) immunoglobulin

    • Effective only in Rh(+) patients with a functional spleen who are DAT negative

    • At doses of 50–75 μg/kg, approximately 80% of Rh(+) children with acute or chronic ITP respond

    • However, there is no clear difference between anti-D and IVIg in the time to reach the end point of achieving a platelet count of 20 × 109/L

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Surgery
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  • Splenectomy

    • Produces a complete response in 70% and partial response in 20% of children

    • Should be considered only after persistence of significant thrombocytopenia for more than 12 months, and the failure of a preferred or alternative second-line therapy

    • Risk of overwhelming infection with encapsulated organisms is ...

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