Orphanet: Congenital factor XI deficiency

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Congenital factor XI deficiency

Disease definition

A rare inherited bleeding disorder characterized by reduced levels and/or activity of factor XI (FXI) resulting in moderate bleeding symptoms, usually occurring after trauma or surgery.


Classification level: Disorder
  • Synonym(s):
    • Hemophilia C
    • PTA deficiency
    • Plasma thromboplastin antecedent deficiency
    • Rosenthal factor deficiency
    • Rosenthal syndrome
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: All ages
  • ICD-10: D68.1
  • OMIM: 612416
  • UMLS: C0015523
  • MeSH: -
  • GARD: 9670
  • MedDRA: -

Detailed information

Article for general public


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