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  • • Autoimmune destruction of platelets

    • Petechiae, ecchymoses, epistaxis, easy bruising

    • No splenomegaly

    • Decreased platelet count, prolonged bleeding time, poor clot retraction, normal coagulation time

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Epidemiology

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  • • The pathogenesis involves a circulating antiplatelet IgG autoantibody; the spleen is both the site of platelet destruction and a significant source of autoantibody production

    • Idiopathic or secondary to the following:

    • -Lymphoproliferative disorder

      -Drugs or toxins

      -Bacterial or viral infection (especially in children)

      -Systemic lupus erythematosus

      -Other conditions

    • The acute form is most common in children, usually occurring before age 8, and often begins 1-3 weeks after a viral upper respiratory tract illness

    • The chronic form, which may start at any age, is more common in women

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Symptoms and Signs

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  • • Ecchymoses or showers of petechiae

    • Bleeding gums

    • Vaginal bleeding

    • GI bleeding

    • Hematuria

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Laboratory Findings

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  • • Platelet count < 100,000/µL

    • Bone marrow shows increased numbers of large megakaryocytes

    • Bleeding time is prolonged

    • Partial thromboplastin time, prothrombin time, and coagulation time are normal

    • Iron deficiency anemia as a result of bleeding

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  • • Symptoms often develop when platelet count is < 50,000/µL

    • Recurrent thrombocytopenia after splenectomy can be evaluated by imaging with indium-labeled platelets to identify accessory spleens

    • Chronic ITP characterized by:

    • -An insidious onset

      -A history of easy bruisability and menorrhagia

      -Showers of petechiae, especially over pressure areas

      -Cyclic remissions and exacerbations, which may continue for several years

    • Specific determinations of antiplatelet antibody titers may aid in diagnosis

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Rule Out

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  • • Other causes of nonimmunologic thrombocytopenia:

    • -Leukemia

      -Aplastic anemia

      -Macroglobulinemia

    • Thrombocytopenia and purpura may be caused by:

    • -Ineffective thrombocytopoiesis (pernicious anemia)

      -Nonimmune platelet destruction (septicemia, disseminated intravascular coagulation, hypersplenism)

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  • • Platelet count

    • Bone marrow biopsy

    • Peripheral blood smear

    • Antiplatelet autoantibody titers if diagnosis remains unclear

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When to Admit

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  • • Severe thrombocytopenia with active bleeding

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When to Refer

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  • • All patients should be managed in conjunction with a hematologist

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  • • Patients with mild or no symptoms need no specific therapy

    • Splenectomy is most effective therapy

    • No platelet transfusions unless actively bleeding

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Surgery

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Indications

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  • • Failure to respond to corticosteroids

    • Relapse after initial remission on corticosteroids

    • Corticosteroid dependence

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Medications

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  • • Corticosteroids

    • Intravenous immunoglobulin (IVIG) for temporary treatment

    • Azathioprine, vincristine

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Treatment Monitoring

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  • • Platelet count

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Complications

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  • • Bleeding

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Prognosis

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  • Splenectomy: Sustained remission in 60-90% of patients

    Corticosteroids: Response in 70-80%; sustained remissions in 20% of adults

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References

George JN et al. Idiopathic thrombocytopenic purpura: diagnosis and management. Am ...

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