Storage Pool Disease

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

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

Prolonged bleeding time

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

Electron microscopy confirms diagnosis

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