dystrophie thrombocytaire hémorragipare

Désignations

Codes : pays ou zone où la désignation a cours (CH, FR, DE, EU = Union européenne, INT = usage international). Une désignation barrée est déconseillée.

Définitions

anglais

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.