Factor XI deficiency--resolving the enigma?
نویسنده
چکیده
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.
منابع مشابه
Factor XI activity and factor XI antigen in homozygous and heterozygous factor XI deficiency.
A relatively potent antiserum against highly purified, unactivated human factor XI antigen was raised in a rabbit. This antiserum, after concentration, neutralized 50% of the factor XI clotting activity of a standard normal plasma at an antiserum dilution of 1/900. The antiserum was used in a neutralization-inhibition assay to study the relation between factor XI clotting activity and factor XI...
متن کاملPrevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency.
Factor XI deficiency, an injury-related bleeding disorder, is rare worldwide but common in Jews in whom 2 mutations, Glu117Stop (type II) and Phe283Leu (type III), prevail. Mean factor XI activities in homozygotes for Glu117Stop and for Phe283Leu are 1 and 10 U/dL, respectively. Inhibitors to factor XI in patients with severe factor XI deficiency have been reported in a small number of instance...
متن کاملHEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency
Factor XI deficiency, an injury-related bleeding disorder, is rare worldwide but common in Jews in whom 2 mutations, Glu117Stop (type II) and Phe283Leu (type III), prevail. Mean factor XI activities in homozygotes for Glu117Stop and for Phe283Leu are 1 and 10 U/dL, respectively. Inhibitors to factor XI in patients with severe factor XI deficiency have been reported in a small number of instance...
متن کاملFactor XI deficiency.
That factor XI has a role in normal blood coagulation is evidenced by the fact that patients with deficiency are prone to excessive bleeding after haemostatic challenge. The role of factor XI in physiological processes has become clearer since the discovery that it is activated by thrombin; this fact has contributed to a revised model of blood coagulation. Factor XI deficiency is particularly c...
متن کاملHigh gene frequency of factor XI (PTA) deficiency in Ashkenazi Jews.
Factor Xi deficiency has previously been observed mainly in Jews. For 34 of 36 probands with factor XI deficiency in Israel, reliable information on ethnic background was obtained. Of 34 probands 33 were of definite Ashkenazi Jewish origin; 1 was of probable Ashkenazi origin. From a survey of factor Xi levels among 428 unrelated healthy Ashkenazi Jews, 35 had partial factor XI deficiency (facto...
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ورودعنوان ژورنال:
- Hematology. American Society of Hematology. Education Program
دوره شماره
صفحات -
تاریخ انتشار 2009