Storage Pool Disease

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

• Bleeding due to defective platelets is classically mucocutaneous, but is not limited to mucocutaneous surfaces

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