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

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Bernard-Soulier syndrome (BSS, named after Jean Bernard and Jean Pierre Soulier) is a 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.

External links

• BSS site

See also

• Gray platelet syndrome
• Glanzmann's thrombasthenia
• von Willebrand disease
• May-Hegglin anomaly