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Platelet function disorders

Platelet function defects comprise a large and heterogeneous group of bleeding disorders that range in severity from mild to severe. Patients may be asymptomatic; however, the majority who are diagnosed present with easy bruising and mucocutaneous bleeding, or with excessive haemorrhage following injury or surgery. As the complex internal biochemical and signal transduction pathways are further understood, and as structural analysis of platelets advances, more of the mechanisms leading to platelet function defects will be uncovered. Despite advances in the understanding of the etiology of these defects in function, treatment remains fairly rudimentary. For platelet function disorders associated with a defect in a plasma coagulation factor, such as vWD and afibrinogenemia, treatment consists of replacement of the deficient coagulation factor. Adjunctive therapies (such as antifibrinolytics, microfibular collagen, fibrin glue, etc.), DDAVP/Stimate, and platelet transfusions remain the mainstay of therapy available at this time. Corticosteroids may play a role in the management of some of these disorders and may lead to a shortening of bleeding time, especially in storage pool defects or release defects failing to respond to DDAVP/Stimate.