Posted by Olga from IP 67.191.249.192 on July 01, 2006 at 13:03:49:
In Reply to: help please posted by med on June 30, 2006 at 09:25:06:
Bernard-Soulier disease- autosomal recessive - is a defective platelet adhesion due to deficiency of Gp Ib receptors on platelets that normally participate in adhesion of platelets to vWF. This disease is characterized by abnormal large platelets and thrombocytopenia, abnormal ristocetin test, increased BT. Patient's condition improves with administration of normal platelets but not with administration of Cryoprecipitate.
vW disease - autosomal dominant - deficiency of vW Factor. Characterized by normal platelets count, increased BT and PTT, decreased Factor VIII:C, Factor VIII:Ag, Factor VIII:vWF, abnormal response to ristocetin. Bleeding time can be corrected with administration of Cryoprecipitate (which is a plasma fraction rich in vWF).