Which disease is associated with a defect in platelet adhesion due to a deficiency in glycoprotein?

Prepare your best for the AAB MT Hematology Exam. Utilize flashcards and multiple choice questions, complete with explanations and hints. Ace the exam!

The disease associated with a defect in platelet adhesion due to a deficiency in glycoprotein is Bernard-Soulier syndrome. This condition is characterized by a deficiency in glycoprotein Ib-IX-V, which plays a crucial role in the interaction between platelets and the von Willebrand factor. This interaction is essential for platelet adhesion to sites of vascular injury, leading to effective hemostasis. In Bernard-Soulier syndrome, the inability of platelets to adhere properly results in bleeding tendencies and can be seen in laboratory findings of thrombocytopenia, where you may also observe large platelets.

Each of the other options represents different hematological disorders. Glanzmann's disease, for example, involves a deficiency in glycoprotein IIb/IIIa and primarily affects platelet aggregation rather than adhesion. Von Willebrand's disease is linked with deficiencies or defects in von Willebrand factor, impacting both adhesion and aggregation but not being caused by a direct defect in the platelet glycoprotein itself. Wiskott-Aldrich syndrome is an immunodeficiency disorder that can affect platelet function but is characterized by a triad of symptoms—thrombocytopenia, eczema, and recurrent infections—and is not primarily a defect in platelet

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