Therapeutic plasma exchange for perioperative management of patients with congenital factor XI deficiency

Abstract

TPE is an effective alternative presurgical hemostatic intervention in HEM-C with potentially lower risk of circulatory volume overload.

Hemophilia C: A Case Report With Updates on Diagnosis and Management of a Rare Bleeding Disorder

“…In a study by Alsammak et al 60% of a cohort of 28 patients with factor XI deficiency underwent surgical procedures without periprocedural hemostatic intervention. They also demonstrated successful therapeutic plasma exchange for four procedures [25]. Topical preparations of anti-fibrinolytics such as ε-amino caproic acid or tranexamic acid can also be used and avoids the systemic side effects such as thromboembolism [3].…”

Perioperative management of a redo aortic root replacement in a patient with severe factor XI deficiency

“…9 Therapeutic plasma exchange has also been reported as a means of avoiding volume overload, which can complicate traditional plasma infusion. 10 The liver appears to be a critical site for FXI production, and FXI deficiency may be corrected by orthotopic liver transplantation. 11,12 Unlike in Hemophilia A and B, routine prophylaxis for everyday activity is not required in individuals with FXI deficiency as they tend not to bleed spontaneously.…”

“…One hundred eighteen patients (88.1%) undergoing major surgery had good or excellent hemostatic control . Therapeutic plasma exchange has also been reported as a means of avoiding volume overload, which can complicate traditional plasma infusion . The liver appears to be a critical site for FXI production, and FXI deficiency may be corrected by orthotopic liver transplantation …”

Acquired factor XI deficiency and therapeutic plasma exchange

“…The risk of functional refractoriness to simple plasma transfusion (or factor concentrates, if available) in inhibitor patients 18,19 makes TPE the favored pre-procedure intervention if there is any significant likelihood of bleeding, as is reflected in ASFA 2016 guidelines. 20 Alsammak et al 1 and Pagano et al 2 have pointed out the benefit of TPE in a subset of congenital FXI deficient patients pre-procedure. Acquired FXI represents an even stronger indication for TPE.…”

“…Both Alsammak et al 1 and Pagano et al 2 have recently reported their use of pre-operative/pre-procedural therapeutic plasma exchange (TPE) for congenital FXI deficiency in the Journal of Clinical Apheresis. We would like to share our experience extending the use of TPE to a case of pregnancy-associated acquired FXI deficiency.…”