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[History of congenital thrombocytic hemorrhagic dystrophy]
1Institut d'hématologie, Hôpital Saint-Louis, Paris, France.
This historical review of the Bernard-Soulier syndrome relates: (1) the first description of the disease; (2) the main data of the research from 1948 to 1982 which led to the discovery of the molecular abnormality (lack or qualitative abnormality of the platelet glycoprotein Ib) responsible for the functional disorder (defective platelet adhesion to the vascular subendothelium) which nowadays defines the disorder.
This historical review of the Bernard-Soulier syndrome relates: (1) the first description of the disease; (2) the main data of the research from 1948 to 1982 which led to the discovery of the molecular abnormality (lack or qualitative abnormality of the platelet glycoprotein Ib) responsible for the functional disorder (defective platelet adhesion to the vascular subendothelium) which nowadays defines the disorder.