Bernard-Soulier syndrome


[Jean A. Bernard, Fr. hematologist, 1907-2006; Jean-Pierre Soulier, Fr. hematologist, 1915-2003]
An autosomal recessive bleeding disorder marked by an inherited deficiency of a platelet glycoprotein. The platelets are large. Bleeding results from defective adhesion of platelets to subendothelial collagen and is disproportionate to the reduction in platelets.

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