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Bernard-Soulier syndrome

Pronunciation: bar-nahr' sul-ya'

Definition:

  1. a coagulation disorder characterized by thrombocytopenia, giant platelets, and a bleeding tendency.
  2. 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

Further information

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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