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Discussion in 'Step I' started by medstu2006, May 18, 2008.
According to FA, the platelet count in Bernard Soulier syndrome is decreased. Does anyone know why?
I always thought that bernard syndrome would have normal platelet counts, its just the bleeding time that would be abnormal b/c of the impaired binding to the endothelium.
Decreased Life Span due to Qualitative defect?
In Glanzmann and Bernard Soulier the bleeding time is increased, but only in Bernard Soulier is the platelet count dec. So im not sure if it's due to a qualitative defect. I dont think Goljan even mentioned about the dec platelet count in Bernard Soulier.
The thrombocytopenia in BS syndrome is generally mild.
They haven't really figured out why though.
And you see abnormally LARGE platelets on peripheral smear.
DOCTORSAIB, you are right.
Bernard-Soulier is a defect in platlet adhesion glycoprotein, the one that can bind to vWB, therefore BS and vWD both giive you a failed ristocetin assay. However, in BS, the ristocetin is not corrigible with addition of normal FVIII/vWB.
The variable thrombocytopenia has been hypothesized to be either an inherent decrease in platelet lifecycle or ineffective thrombopoiesis.
Clinically, BS shows heterogeneity; variants include an antibody-mediated (against the glycoprotein receptor), called pseudo-bernard soulier. And even a case associated with Gaucher's Dz, which presented with a BS-like picture, including abnormal ristocetin. It was found patient had intact GPIb receptor and no antibodies detected. This was termed pseudo-pseudo-Bernard-Soulier.
Glanzmann's dz is a defect in platlet GP IIb/IIIa, for aggregation. Variants have also included an antibody-mediated type.