What are the ethnic and racial predilections of hemophilia C (deficiency of factor XI)?

Updated: Dec 18, 2020
  • Author: Vineeta Gupta, MD, DNB, MAMS, FRCPCH, FUICC; Chief Editor: Hassan M Yaish, MD  more...
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Answer

Internationally, deficiency of factor XI is reported in most racial groups, with the highest frequency in persons of Ashkenazi or Iraqi Jewish descent [10, 11] ; in Israel, the estimated rate for heterozygosity is 8%. In the United Kingdom national database, 1696 patients (many of whom were non-Jewish) with factor XI deficiency were registered in a population of about 60 million (data for 2006), but most of these have partial deficiency [12] ; factor XI deficiency is more common than factor IX deficiency (hemophilia B). In the French Basque country (home to the most ancient ethnic group of Western Europe, the Basques), 39 patients were identified among the general population of 290,000. [13]

A study from Austria, by Gebhart et al, found that out of 418 patients with mild to moderate bleeding disorders, at least 3 (0.7%) had factor XI deficiency, compared with 11 (2.6%), 3 (0.7%), and 1 (0.2%) with factor VIII, IX, and XIII deficiency. [14]


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