Dataset Information

Concomitant use of bypassing agents with emicizumab for people with haemophilia A and inhibitors undergoing surgery.

ABSTRACT:

Introduction

Surgery in people with haemophilia and factor VIII inhibitors is typically managed with perioperative administration of haemostatic agents to prevent or control the occurrence of bleeding events. Practical experience of surgery in patients with inhibitors who are receiving treatment with emicizumab is growing; however, the novelty of the situation means that standardised guidelines are lacking with regard to the concomitant administration of haemostatic agents, including dose and laboratory monitoring.

Aim

To review approaches to haemostatic management during major and minor invasive procedures in patients with haemophilia A and inhibitors, and to provide recommendations for controlling bleeding events.

Methods

A search was conducted, limited to the past 4 years (January 2016-April 2020), pertaining to published evidence of surgery for patients receiving emicizumab. Publications identified from the search were manually reviewed to determine studies and case reports relevant for inclusion.

Results

Identified literature and practical experience of the authors were used to build a consensus of practical recommendations for the concomitant administration of haemostatic agents during the perioperative period for elective surgery in patients with inhibitors who are receiving emicizumab.

Conclusions

The current evidence base indicates that surgery can be successfully performed in patients with inhibitors who are receiving emicizumab and that bypassing agents can be used concomitantly. Data from prospective studies are required to further support recommendations for haemostatic management of surgery in patients receiving emicizumab.

SUBMITTER: Jimenez-Yuste V

PROVIDER: S-EPMC8359827 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundAcquired haemophilia A is a rare bleeding disorder caused by the development of specific autoantibodies against coagulation factor VIII. Standard treatment, usually steroids alone, or in combination with cyclophosphamide, aims to stop acute bleeds by using haemostatic agents to promote clotting. Rituximab may be an alternative approach to the treatment of acquired haemophilia by eradicating FVIII autoantibodies. This is an update of a previously published Cochrane Review.ObjectivesTo assess the efficacy and adverse effects of rituximab for treating people with acquired haemophilia A.Search methodsWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's trials registers, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and conference proceedings (January 2021). We also undertook searches of CENTRAL, MEDLINE and online trial registries (January 2021).Selection criteriaRandomised and quasi-randomised controlled trials of rituximab for people with acquired haemophilia A, with no restrictions on gender, age or ethnicity.Data collection and analysisNo trials matching the selection criteria were eligible for inclusion.Main resultsNo trials matching the selection criteria were eligible for inclusion.Authors' conclusionsWe found no randomised clinical trials of rituximab for acquired haemophilia A. Thus, we are not able to draw any conclusions or make any recommendations on rituximab for eradicating inhibitors in people with acquired haemophilia A based on the highest quality evidence. Given that undertaking randomised controlled trials in this field is a complex task, we suggest that, while planning such trials, clinicians treating the disease continue to base their choices on alternative, lower-quality sources of evidence. In a future update of this review, we plan to appraise and incorporate eligible randomised controlled trials, as well as other high-quality, non-randomised studies.