Glanzmann disease is characterised by which of the following?: (A) Congenital defect of RBCs, (B) Defect of neutrophils, (C) Congenital defect of platelets, (D) Clotting factor deficiency
Answer is C. Glanzmann disease - AR inheritance - Inherited defect in glycoprotein GpIIb-IIIa The GpIIb/IIIa receptors on activated platelets form bridging crosslinks with fibrinogen, leading to platelet aggregation. - Functional platelet defect as they are less able to adhere to each other and to damaged blood vessel. S/S- purpura, epistaxis, gingival hemorrhage, and menorrhagia are nearly constant features; gastrointestinal bleeding and hematuria are less common. - Increased bleeding with Normal platelet count - ADP and collagen aggreagtion tests are done for aggregation defect.