• Factor XI deficiency: mainly found in Ashkenazi Jews, recessive inheritance. Homozygotes <5% levels of factor XI. Factor XI concentrate given.
  • Factor VII deficiency: autosomal recessive, variable bleeding severity, risk of CNS haematoma. Diagnosis by factor VII assay, treatment with recombinant factor VII
  • Factor V deficiency: treated with virally inactivated fresh frozen plasma
  • Factor XIII deficiency: severe haemorrhagic tendency, poor healing (profuse bleeding from umbilical cord). Clots soluble in urea (failure of cross-linking). Factor XIII concentrate given.
  • Fibrinogen: abnormalities may be quantitative (apofibrinogennaemia and hypofibrinogenaemia) or qualitative (dysfbrinogenaemia).
  • Apofibrinogennaemia: autosomal recessive, failure to clot in any test of coagulation, severe bleeding tendency
  • Dysfibrinogenaemia: rare group of autosomal dominant conditions, haemorrhagic or thrombotic states

Related entries