Tags: Disease.
Bernard–Soulier syndrome (BSS) also called hemorrhagiparous thrombocytic dystrophy is a rare autosomal recessive coagulopathy (bleeding disorder) that causes a deficiency of glycoprotein Ib (GpIb) the receptor for von Willebrand factor which is important in clot formation. The incidence is estimated to be less than 1 in 1 million persons based on cases reported from Europe North America and Japan.It is a Giant Platelet Syndrome that is characterized by abnormally large platelets.