Factor XI deficiency--resolving the enigma?
The management of factor XI deficiency is not straightforward for three reasons: firstly, the role of this factor in the coagulation pathway is not clearly understood; secondly, the bleeding tendency, although mild, is unpredictable and does not clearly relate to the factor XI level; and thirdly, all treatment products, although available, have some potentially serious side effects. These factors (or enigmas) contribute to the variable management of patients with this coagulation factor deficiency, but recent research is helping to clarify some of these areas.
Bolton-Maggs
Paula H B
PH
Department of Clinical Haematology, Manchester Royal Infirmary, Manchester, United Kingdom. paula.bolton-maggs@manchester.ac.uk
D000818:Animals; D025901:Carboxypeptidase B2 / Q000502:physiology; D019281:Dimerization; D004789:Enzyme Activation; D005006:Ethnicity / Q000235:genetics; D015942:Factor VIIa / Q000627:therapeutic use*; D005172:Factor XI / Q000737:chemistry / Q000276:immunology / Q000502:physiology / Q000627:therapeutic use*; D005173:Factor XI Deficiency / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000208:ethnology / Q000235:genetics; D005260:Female; D006801:Humans; D007518:Isoantibodies / Q000276:immunology; D007585:Jews / Q000235:genetics; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D008958:Models, Molecular; D019683:Penetrance; D010949:Plasma; D019106:Postoperative Hemorrhage / Q000209:etiology / Q000517:prevention & control; D006473:Postpartum Hemorrhage / Q000209:etiology / Q000517:prevention & control; D011247:Pregnancy; D011487:Protein Conformation
D007518:Isoantibodies; D005172:Factor XI; D025901:Carboxypeptidase B2; D015942:Factor VIIa