Bernard-Soulier syndrome
Pronunciation: bār-nahr′ sūl-yā′
Definition:
- a coagulation disorder characterized by thrombocytopenia, giant platelets, and a bleeding tendency.
- an autosomal recessive disorder of absent or decreased platelet membrane glycoproteins Ib, IX, and V (the receptor for factor VIII R). This deficiency can lead to a failure to bind von Willebrand factor, causing moderate bleeding.
Synonym(s): Bernard-Soulier disease
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