Bernard-Soulier Syndrome

Explore an in-depth video lecture on Bernard-Soulier Syndrome (BSS), an autosomal recessive disorder affecting platelet function. Learn about the defective GP1b platelet receptor, its impact on primary hemostasis, and the characteristic abnormalities in laboratory tests such as Ristocetin Cofactor Assay (RIPA) and prolonged bleeding time. Discover the unique feature of macrothrombocytopenia in BSS patients and understand the importance of platelet count and bleeding time in diagnosing platelet disorders. Gain insights into the causes, aggregation issues, and potential complications like cranial bleeding associated with BSS. Enhance your understanding of hematological disorders and their clinical implications through this comprehensive exploration of Bernard-Soulier Syndrome.