https://orcid.org/0000-0002-8329-8409
Abstract
Factor XI (FXI) deficiency is a rare bleeding disorder that presents complex challenges in patient assessment and bleeding risk management. Despite generally causing mild to moderate bleeding symptoms, clinical manifestations can vary, and bleeding tendency does not always correlate with FXI plasma levels or genotype. Our manuscript delves into the age-related nuances of FXI deficiency across an individual's lifespan. We emphasize issues faced by specific groups, including neonates and females of reproductive age experiencing abnormal uterine bleeding and postpartum hemorrhage. Older patients present unique challenges and concerns related to the management of bleeding as well as thrombotic complications. The current assortment of diagnostic laboratory assays shows limited success in predicting bleeding risk in the perisurgical setting of patients with FXI deficiency. This review explores the intricate interplay between individual bleeding profiles, surgical sites, and FXI activity levels. We also evaluate the accuracy of existing laboratory assays in predicting bleeding and discuss the potential role of investigational global assays in perioperative assessment. Furthermore, we outline our suggested diagnostic approach to refine treatment strategies and decision making. Available treatment options are presented, including antifibrinolytics, replacement products, and recombinant activated FVII. Finally, we discuss promising nonreplacement therapies for the treatment of rare bleeding disorders that can potentially address the challenges faced when managing FXI deficiency–related bleeding complications.
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Comments
The Other Side of The Coin: From The Management of Factor 11 deficiency to Factor 11 Inhibitors
Submitted:
January 27, 2024
Response to:
Factor XI Deficiency: Phenotypic Age related considerations and Clinical Approach Towards Bleeding Risk Assessment
Author(s)
Dong Liu; Rui Liang; Xiaomeng Zhang; Zhenguo Zhai
Affiliation(s)
Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Institute of Respiratory Medicine Beijing,
Disclosure
None Reported
Comment Title
The Other Side of The Coin: From The Management of Factor 11 deficiency to Factor 11 Inhibitors
Comment Body
We read with interest this study of high clinical value. We come from a venous thromboembolism（VTE）research team focused on risk assessment and prevention, and we are very interested in FXI deficiency and FXI inhibitors.
Several recent studies have confirmed the efficacy and safety of factor Ⅺ inhibitors in the prophylaxis of VTE in patients after knee arthroplasty. In addition, studies of coagulation factor Ⅺ inhibitors in people at high bleeding risk have further confirmed their safety just like patients with renal insufficiency and concomitant antiplatelet therapy. Despite the advantages of coagulation factor Ⅺ inhibitors, there are still some limitations. Since no solid evidence
sof correlation between bleeding symptoms and coagulation factor Ⅺ activity or genotype have been found, and traditional coagulation assays were not effective for predicting?major bleeding. So how to assess the bleeding risk is still an unavoidable clinical problem in the application of coagulation factor Ⅺ inhibitors.Assaf Arie Barg and his colleagues propose such a diagnostic approach and evaluate the accuracy of existing laboratory tests in predicting bleeding. As the application of FXI factor inhibitors
increases in the future, whether this assessment could be applied to patients with FXI inhibitors or how this approach could contribute to the management of bleeding in this special group deserves more thoughts. We should make full use of the available evidencesof FXI deficiency to guide the use of FXI inhibitors.As the authors mentioned, further research is required to develop more effective tools for assessing bleeding risk of FXI deficiency, and in patients treated with FXI inhibitors. More prospective clinical studies and validation studies are warranted for these two groups.
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