The management of factor XI deficiency.
Factor XI deficiency leads to a more variable bleeding tendency than haemophilia A or B. Although severely deficient individuals are likely to bleed excessively especially after surgery in areas of the body with increased fibrinolysis, there is evidence that some partially deficient individuals are at risk of excessive bleeding. This will entail careful planning for surgery. Several therapeutic modalities are available which include fresh frozen plasma, factor XI concentrates, fibrin glue, antifibrinolytic drugs and desmopressin. The advantages and risks of these are considered. Factor XI concentrate may be indicated for procedures with a significant risk of bleeding especially in younger patients with severe deficiency, but its use in older patients has been associated with thrombotic phenomena. If fresh frozen plasma is to be used, it is preferable to obtain one of the virally inactivated products. Fibrin glue is a useful treatment which deserves further study.
Bolton-Maggs
P H
PH
Department of Haematology, Alder Hey Children's Hospital, Liverpool, UK. P.H.Boltonmaggs@liv.ac.uk
D005172:Factor XI / Q000627:therapeutic use; D005173:Factor XI Deficiency / Q000628:therapy*; D015718:Fibrin Tissue Adhesive / Q000627:therapeutic use; D006801:Humans; D010951:Plasma Exchange
D015718:Fibrin Tissue Adhesive; D005172:Factor XI