Immune thrombocytopenia (ITP)
Immune thrombocytopenia is an acquired isolated thrombocytopenia from antiplatelet autoantibody-mediated destruction plus impaired marrow production, presenting with mucocutaneous bleeding and no splenomegaly. It is a diagnosis of exclusion made by a normal smear and normal other cell lines, and treatment is driven by bleeding risk, starting with glucocorticoids with or without IVIG or anti-D.
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What this article covers
- Overview
Definition: immune thrombocytopenia (ITP), formerly idiopathic thrombocytopenic purpura, is an acquired autoimmune disease in which antiplatelet autoantibodies (usually against glycoprotein IIb/IIIa)…
- Epidemiology
Bimodal pattern: a childhood peak, most often acute and post-viral, and an adult pattern that is more often chronic and insidious.
- Risk factors
Recent viral infection (children): an antecedent viral illness 1 to 4 weeks before onset is the dominant antecedent in pediatric acute ITP.
- Pathophysiology
Autoantibody formation: IgG autoantibodies form against platelet surface glycoproteins, most often glycoprotein IIb/IIIa, coating circulating platelets.
- Clinical features
Isolated mucocutaneous bleeding: petechiae (nonblanching, most prominent on the lower extremities), purpura, ecchymoses, and epistaxis or gum bleeding; spontaneous bleeding becomes more likely below 10,000 to 20,000/microL.
- Differential diagnosis
Thrombotic thrombocytopenic purpura (TTP): microangiopathic hemolytic anemia, schistocytes, fever, neurologic changes, and renal dysfunction; ADAMTS13 activity below 10% confirms TTP and demands…
- Diagnosis
ITP is a diagnosis of exclusion; there is no confirmatory test.
- Management
Base treatment on platelet count and bleeding, not diagnosis alone: counts of at least 50,000/microL do not routinely require treatment; asymptomatic patients or minor mucocutaneous bleeding with a…
- Complications
Intracranial or other major hemorrhage: rare but the most feared complication, predominantly at counts under 10,000 to 20,000/microL.
- Prognosis
Excellent in children: most childhood ITP resolves spontaneously within 6 to 12 months without long-term sequelae.
- Prevention
No primary prevention exists for primary ITP; risk reduction centers on preventing complications of severe thrombocytopenia and of treatment.
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