Preoperative management of factor XI deficiency with therapeutic plasma exchange: A case report and literature review

Abstract

Patients with factor XI deficiency may have bleeding complications during surgery. Because bleeding severity and factor levels correlate poorly, factor replacement needs to be personalized based on bleeding history and type of procedure. We report a 65-year-old male with factor XI deficiency (7 IU dL^-1 ) who presented before scheduled hip arthroplasty. He had a history of total hip arthroplasty complicated by bleeding, delayed healing and prosthesis removal, despite receiving prophylactic treatment with plasma infusion. For the current surgery a factor XI ≥50 IU dL^-1 level was targeted. The calculated plasma infusion needed to achieve this goal was 3100 mL (14 U). Because of concerns about circulatory overload and inability to achieve target by simple infusion, prophylactic treatment with therapeutic plasma exchange (TPE) was requested. TPE was performed the morning before the surgery, using 100% plasma as replacement fluid (3912 mL of plasma), and a positive fluid balance of 631 mL. Factor XI activity level was 51 IU dL^-1 immediately post TPE. The patient received daily infusions of 3 U (∼ 660 mL) of plasma to maintain a factor XI level of 30 IU dL^-1 until post-operative day 7. Aminocaproic acid was given during the surgery and until post-operative day 10. There were no bleeding or thrombotic complications.

Conclusion: TPE was effective in increasing factor XI levels; it was well tolerated and did not result in circulatory overload. TPE can be considered when therapeutic factor levels cannot be achieved by simple plasma infusion, or when circulatory overload is a concern. J. Clin. Apheresis 31:579-583, 2016. © 2015 Wiley Periodicals, Inc.

Keywords: apheresis; exchange; factor XI deficiency; hemophilia.