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Review
. 2021 Dec;35(6):1157-1169.
doi: 10.1016/j.hoc.2021.07.012. Epub 2021 Sep 15.

Factor XI Deficiency

Magdalena Dorota Lewandowska  1 , Jean Marie Connors  2
Affiliations
Free article
Review

Factor XI Deficiency

Magdalena Dorota Lewandowska et al. Hematol Oncol Clin North Am. 2021 Dec.
Free article

Abstract

Factor XI (FXI) deficiency (hemophilia C or Rosenthal disease) was first described in the 1950s in a multigenerational family experiencing bleeding related to surgery and dental procedures. Managing patients with FXI deficiency presents several challenges, including a lack of correlation of bleeding symptoms with FXI activity levels, the large volume of fresh frozen plasma required to achieve hemostatic FXI levels, lack of availability of FXI concentrate in certain regions of the world, and the inherent thrombotic risk associated with replacement therapy. This article summarizes presentation, diagnosis, and management of patients with FXI deficiency in a variety of clinical settings.

Keywords: Factor XI deficiency; Hemophilia C; Plasma thromboplastin antecedent deficiency; Rosenthal syndrome.

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Conflict of interest statement

Disclosure M.D. Lewandowska: Advisory board for Bio Products Laboratory. All Honoraria donated to the Indiana Hemophilia and Thrombosis Center. J.M. Connors: No relationships to disclose related to this article; others include personal fees for scientific Ad Boards and Consulting: Abbott, Anthos, Alnylam, Bristol-Myers Squibb, Portola, and Takeda. Research funding to the institution from CSL Behring.

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