Bernard-Soulier syndrome
Bernard-Soulier syndrome | |
Bernard-Soulier syndrome. (Image courtesy of Melih Aktan M.D.) | |
ICD-10 | D69.1 |
ICD-9 | 287.1 |
OMIM | 231200 |
DiseasesDB | 1356 |
eMedicine | ped/230 |
MeSH | D001606 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Bernard-Soulier syndrome (BSS, named after Jean Bernard and Jean Pierre Soulier) is an inherited medical condition. this condition is a disorder of the vWF (vonWillebrand factor) receptor termed glycoprotein Ib(GpIb).
It presents as a bleeding disorder due to the inability of platelets to bind and aggregate at sites of vascular endothelial injury.