Article
Bernard-Soulier syndrome (hemorrhagiparous thrombocytic dystrophy).
Orphanet journal of rare diseases - 16 Nov 2006
Lanza François
Abstract excerpt
Bernard-Soulier syndrome (BSS), also known as Hemorrhagiparous thrombocytic dystrophy, is a hereditary bleeding disorder affecting the megakaryocyte/platelet lineage and characterized by bleeding tendency, giant blood platelets and low platelet counts. This syndrome is extremely rare as only approximately 100 cases have been reported in the literature. Clinical manifestations usually include purpura, epistaxis,...
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