The rare coagulation disorders--review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

Haemophilia. 2004 Sep;10(5):593-628. doi: 10.1111/j.1365-2516.2004.00944.x.


The rare coagulation disorders are heritable abnormalities of haemostasis that may present significant difficulties in diagnosis and management. This review summarizes the current literature for disorders of fibrinogen, and deficiencies of prothrombin, factor V, FV + VIII, FVII, FX, the combined vitamin K-dependent factors, FXI and FXIII. Based on both collective clinical experience and the literature, guidelines for management of bleeding complications are suggested with specific advice for surgery, spontaneous bleeding, management of pregnancy and the neonate. We have chosen to include a section on Ehlers-Danlos Syndrome because haematologists may be consulted about bleeding manifestations in such patients.

Publication types

  • Review

MeSH terms

  • Afibrinogenemia / diagnosis
  • Afibrinogenemia / genetics
  • Afibrinogenemia / therapy
  • Blood Coagulation Disorders / diagnosis
  • Blood Coagulation Disorders / therapy*
  • Clinical Laboratory Techniques
  • Coagulation Protein Disorders / diagnosis
  • Coagulation Protein Disorders / therapy
  • Hemorrhagic Disorders / etiology
  • Hemorrhagic Disorders / therapy
  • Humans
  • Hypoprothrombinemias / diagnosis
  • Hypoprothrombinemias / therapy
  • Practice Guidelines as Topic*
  • Vitamin K Deficiency / diagnosis
  • Vitamin K Deficiency / therapy