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Congenital Factor XI Deficiency

  • Tahere Tabatabaei
  • Akbar Dorgalaleh
Chapter

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.

Keywords

Factor XI deficiency Rare bleeding disorder Diagnosis Treatment Inhibitor 

Notes

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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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Tahere Tabatabaei
    • 1
  • Akbar Dorgalaleh
    • 1
  1. 1.Department of Hematology and Blood transfusionSchool of Allied Medicine, Iran University of Medical SciencesTehranIran

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