Bernard Soulier Syndrome – MCQ

False regarding Bernard Soulier Syndrome?
A. Large platelets
B. Decreased platelet count
C. Ristocetin aggregation normal
D. Platelet aggregation in response to collagen and ADP is normal

Correct answer : C. Ristocetin aggregation normal

In Bernard Soulier Syndrome, platelet response to ADP, collagen and Thrombin are normal.
But no aggregation occurs in response to Ristocetin.
It occurs due to defect in glycoprotein Ib – the receptor for von Willebrand factor

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