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Congenital Factor XI Deficiency

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Congenital Bleeding Disorders

Abstract

Factor XI (FXI) deficiency is a rare autosomal recessive bleeding disorder due to F11 gene defects. Estimated incidence of disorder is 1 per 1 million in the general population, but in special populations such as Ashkenazi Jews, the prevalence is 1 per 450 individuals. FX deficiency is a mild bleeding disorder, mostly with post-traumatic or post-surgical hemorrhages. Based on FXI antigen and activity levels, the disease is classified in two types: the more frequent cross-reactive material negative (CRM−) and the rare cross-reactive material positive (CRM+). Different therapeutic choices are available including fresh frozen plasma (FFP) and FXI concentrates that can provoke inhibitor formation, while recombinant FVII can be used for such patients.

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Acknowledgment

We appreciate the valuable work of Maryam Tabatabaei Shoja on this chapter that significantly improved the quality of this chapter.

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  1. Department of Hematology and Blood transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran

    Tahere Tabatabaei & Akbar Dorgalaleh

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  1. Tahere Tabatabaei
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    Akbar Dorgalaleh

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Tabatabaei, T., Dorgalaleh, A. (2018). Congenital Factor XI Deficiency. In: Dorgalaleh, A. (eds) Congenital Bleeding Disorders . Springer, Cham. https://doi.org/10.1007/978-3-319-76723-9_12

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