Idiopathic Thrombocytopenic Purpura

Idiopathic
Thrombocytopenic
Purpura (ITP)
Feature

Roger S. Riley, M.D., Ph.D.

April, 2006

Disease Facts

Synonyms

ITP, primary immune thrombocytopenic purpura, autoimmune thrombocytopenic purpura.

Epidemiology

True incidence is unknown, since individuals with mild disease may be asymptomatic
and undiagnosed. In the United States, symptomatic disease occurs in about 70
adults/1,000,000 and 50 children/1,000,000.

Etiology &
Pathogenesis

Autoimmune disease, with anti-platelet antibody formation (usually specific for the
platelet GPIIbIIIa receptor) and peripheral platelet destruction. Acute ITP usually occurs in children, follows an acute viral infection or recent live virus immunization, and
usually undergoes a spontaneous remission within two months. Chronic ITP is more
common in adults, shows an insidious presentation, and lasts > 6 months. The pathogenesis is complex and multifactorial, including a failure of self-antigen recognition and
tolerance, altered cytokine secretion, impaired megakarypoiesis, and impaired cellmediated cytotoxicity.

Clinical
Presentation

Symptomatic individuals present with platelet-type bleeding problems. These

include non-palpable petechiae, purpura, epistaxis, gingival bleeding, menorrhagia, and easy bruising. GI bleeding, hematuria, retinal hemorrhage, and
even intracranial hemorrhage can occur in severe cases. Splenomegaly is unusual.

Differential
Diagnosis

All causes of thrombocytopenia must be excluded. The major considerations

include pseudothrombocytopenia, DIC, viral infections (especially HIV and
EBV), TTP, and drug-induced thrombocytopenia (heparin, alcohol, sulfinamides,
quinine, quinidine). Other disease in the differential include pregnancyassociated thrombocytopenia, autoimmune disease (especially lupus erythrmatous), other infections, liver disease, hematologic and other malignancies,
transfusion reaction.

Laboratory
Features

Laboratory studies are more valuable in the exclusion of other diseases than in
providing definitive evidence of ITP. Variable thrombocytopenia with a normal
white blood count and erythrocytic indices (in the absence of severe hemorrhage). Peripheral blood smear examination reveals thrombocytopenia with
normal leukocytic and erythrocytic morphology. Coagulation assays are within
normal limits. Bone marrow evaluation reveals normal to increased megakaryocytes with normal morphology. Assays for platelet-associated immunoglobulin
by ELISA or flow cytometry may be positive, but these assays are presently
considered unreliable. Pseudothrombocytopenia must be excluded by peripheral
smear examination for platelet clumping. The exclusion of other causes of
thrombocytopenia should include other laboratory assays, especially a CBC, Ddimer and FDP assay, LDH, viral serology (especially for HIV), anti-nuclear antibody, rheumatoid factor studies, and ESR, liver function studies, and pregnancy test.

Idiopathic Thrombocytopenic Purpura

Feature

Disease Facts

Treatment

Supportive care, including platelet transfusions, must be provided until remission occurs or treatment is effective. No specific treatment is usually provided
for children with platelet counts >30,000/L or adults with >50,000 platelets/
L. Intravenous immunoglobulin (IVIG, IV Rh immune globulin, IV anti-Rh(D)
and corticosteroids are the first line of therapy for patients with severe thrombocytopenia. Rituximab is often used in patients with refractory ITP.

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Idiopathic Thrombocytopenic Purpura

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