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What is Bernard-Soulier syndrome is caused by the deficiency of and explain in detail?
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