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Platelet Function Disorders
Platelets are blood cells responsible for blood clotting, also known as hemostasis. When their count is normal but their function is impaired, platelet function disorders (thrombocytopathies) occur. In such cases, platelets fail to adhere properly to each other or to the damaged vessel wall, preventing the formation of a stable clot.
Glanzmann Thrombasthenia
This is a hereditary disorder caused by the absence or dysfunction of specific platelet surface proteins (GP IIb/IIIa), which are required to bind fibrinogen and mediate platelet aggregation. As a result, platelets cannot form a stable clot.
Bernard-Soulier Syndrome
This is also an inherited disorder resulting from defects in another protein complex (GP Ib-IX-V), which normally binds platelets to von Willebrand factor, enabling their adhesion to the site of vascular injury. In this condition, platelets are typically abnormally large.
Clinical Manifestations
Both disorders present with mucocutaneous bleeding, including easy bruising, recurrent epistaxis, and gingival bleeding, and in women, menorrhagia.
Management
Treatment focuses on the prevention and control of bleeding episodes, mainly through platelet transfusion.
In addition to platelet transfusion, other therapeutic options include:
Antifibrinolytic agents (e.g., tranexamic acid) help stabilize existing clots.
In severe, life-threatening bleeding, recombinant activated factor VII (rFVIIa) may be used. This agent bypasses the platelet defect and directly activates coagulation.
