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An autosomal recessive disorder characterized by giant platelets with membranes lacking glycoprotein Ib, the probable receptor for plasma von Willebrand's factor; this keeps the platelets from binding the factor, which is necessary for their adhesion to the subendothelial surfaces of blood vessels. Symptoms include mild to moderate mucocutaneous and visceral hemorrhaging, purpura, and prolonged bleeding time.  1, fiche 1, Anglais, - Bernard%2DSoulier%20syndrome

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En pathologie, la glycoprotéine Ib manque dans la dystrophie thrombocytaire hémorragipare, ou syndrome de Jean Bernard-Soulier avec trouble de l'adhésivité plaquettaire .  1, fiche 1, Français, - dystrophie%20thrombocytaire%20h%C3%A9morragipare

Fiche 1,  Justifications,  Espagnol