What condition is characterized by a bleeding disorder due to GpIb deficiency?

The condition characterized by a bleeding disorder due to GpIb deficiency is Bernard-Soulier disease. This disorder is a rare inherited condition caused by a defect in the GpIb-IX-V complex on platelet membranes, which is essential for the binding of von Willebrand factor (vWF) to platelets. This interaction is critical for platelet adhesion to the damaged vascular endothelium, particularly under shear stress conditions that occur in blood flow.

In individuals with Bernard-Soulier disease, the absence or dysfunction of GpIb results in ineffective platelet aggregation and ultimately leads to a bleeding tendency, often presenting with mucocutaneous bleeding such as easy bruising, epistaxis, and menorrhagia. Laboratory findings typically show thrombocytopenia (low platelet count), large platelets (macrothrombocytes), and impaired platelet aggregation in response to ristocetin due to the lack of functional GpIb.

Other options listed pertain to different bleeding disorders with distinct pathophysiologies: Wiskott-Aldrich syndrome involves immune deficiency and eczema alongside thrombocytopenia, Hemophilia A is characterized by a deficiency of factor VIII in the coagulation cascade, and Glanzmann thrombasthenia involves a deficiency of