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Bernard-Soulier syndrome

What is Bernard-Soulier syndrome?

Bernard Soulier syndrome (BSS) is an inherited platelet disorder characterized by mild to severe bleeding tendency , macrothrombocytopenia and absent ristocetin-induced platelet agglutination.

Other condition names

  • Giant platelet syndrome
  • Hemorrhagiparous thrombocytic dystrophy

Inheritance type

Autosomal dominant, Autosomal recessive

Prevalence

  • Worldwide: <1 in 1,000 000

Age of Onset

  • All ages
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.