Bernard-Soulier syndrome is caused by the deficiency of: (A) Glycoprotein complex Ib-IX, (B) Glycoprotein complex IIb-IIIa, (C) Glycoprotein complex Ib-IIIa, (D) Glycoprotein complex IIb-IX
Answer is A. Bernard-Soulier syndrome is caused by an inherited deficiency of the platelet membrane glycoprotein complex Ib-IX. This glycoprotein is a receptor for vWF and is essential for normal platelet adhesion to the subendothelial extracellular matrix. Affected patients have a variable, often severe, bleeding tendency.Ref: Robbins and Cotran Pathologic Basis of Disease; 9th edition; Chapter 14; Red Blood Cell and Bleeding Disorders; Page no: 660