TERMIUM Plus®

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BERNARD-SOULIER SYNDROME [1 record]

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Record 1 2011-01-05

English

Subject field(s)
  • Blood
  • Symptoms (Medicine)
DEF

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.

French

Domaine(s)
  • Sang
  • Symptômes (Médecine)
CONT

En pathologie, la glycoprotéine Ib manque dans la dystrophie thrombocytaire hémorragipare, ou syndrome de Jean Bernard-Soulier avec trouble de l'adhésivité plaquettaire .

Key term(s)
  • maladie de Jean Bernard-Soulier

Spanish

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