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Key Features

  • Group of mild bleeding disorders caused by defects in release of alpha or dense (delta) platelet granules, or both

Clinical Findings

  • Variable bleeding, ranging from spontaneous to mild and trauma-related bruising to postoperative bleeding

Diagnosis

  • Prolonged bleeding time

  • Platelet aggregation studies characteristically show platelet dissociation following an initial aggregatory response

  • Electron microscopy confirms diagnosis

Treatment

  • Mainstay of treatment (including periprocedural prophylaxis) is transfusion of normal platelets

  • Desmopressin acetate (DDAVP), antifibrinolytic agents, and recombinant human activated factor VII also have been used successfully

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