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Coagulation factor deficiency or dysfunction

Inherited

Haemophilia A (factor VIII deficiency) — X-chromosome linked bleeding disorder that occurs primarily in males. The first bleeding episode may be with circumcision or other procedure as an infant. The severity of the bleeding caused by a factor VIII deficiency depends on its activity level (how well it is functioning). If it is very low it may cause severe life threatening bleeding, if it is moderate it may only cause mild to moderate bleeding – becoming an issue primarily when having surgery or dental procedures.

von Willebrand’s disease — the commonest bleeding disorder, resulting from reduced (type 1) or dysfunctional (type 2) or absent (type 3) von Willebrand factor. von Willebrand factor is a protein that adheres platelets to the site of a blood vessel injury. It is associated with factor VIII: if VIII is deficient then vWF may be affected.

Other factor deficiencies — II, V, VII, IX, X, (Haemophilia B, also called Christmas disease), all much less common.

Acquired:

  • Liver dysfunction or disease (because the liver synthesises factors II, VII, IX and X)
  • Vitamin K deficiency
  • Fat malabsorption
  • Snake venom (produces intravascular activation of clotting with consequent consumption of clotting factors)
  • Therapy for bone marrow and myeloproliferative disorders
  • Factor inhibitors (antibodies that target a specific clotting factor, such as factor VIII, decreasing its level and making factor replacement more of a challenge)
  • Anticoagulant drugs: such as coumadin (warfarin) or heparin, these drugs are used to treat clotting disorders but in excessive amounts may cause bleeding
  • Some bacterial infections
  • DIC (disseminated intravascular coagulation), may cause both bleeding and clotting. It is usually an acute condition, for example from a complicated childbirth – as a result of uterine tissue contacting the blood stream, from an endotoxin produced during a severe infection, or due to certain cancers such as leukaemia. DIC causes tiny clot formation throughout the body, using up clotting factors at an accelerated rate – leading to excessive bleeding.

Last Review Date: May 1, 2018