Management of bleeding and invasive procedures in haemophilia A patients with inhibitor treated with emicizumab (Hemlibra<sup>®</sup>): Proposals from the French network on inherited bleeding disorders (MHEMO), the French Reference Centre on Haemophilia, in collaboration with the French Working Group on Perioperative Haemostasis (GIHP)

Susen, Sophie; Gruel, Yves; Godiér, Anne; Harroche, Annie; Chambost, Hérvè; Lasne, Dominique; Rauch, Antoine; Roullet, Stéphanie; Fontana, Pierre; Goudemand, Jenny; Maistre, Emmanuel de; Chamouard, V.; Wibaut, B.; Albaladéjo, Pierre; Négrier, Claude

doi:10.1111/hae.13817

Cited by 44 publications

(73 citation statements)

References 18 publications

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“…Optionally, tranexamic acid can be given as a supportive measure to rFVIIa. 81 Further, the Italian Association of Haemophilia Centres (AICE) have provided a practical guidance paper on how to manage patients with hemophilia A and inhibitors under treatment with emicizumab in the emergency department. 82 However, the uncritical, permissive use of rFVIIa in emicizumab-treated patients is unwarranted, since the results of the bleeding analysis from HAVEN 1, 2, and 4 show some major limitations.…”

Section: Bleeding Under Treatment With Emicizumabmentioning

confidence: 99%

Bridging the Missing Link with Emicizumab: A Bispecific Antibody for Treatment of Hemophilia A

et al. 2020

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Hemophilia A, characterized by absent or ineffective coagulation factor VIII (FVIII), is a serious bleeding disorder that entails severe and potentially life-threatening bleeding events. Current standard therapy still involves replacement of FVIII, but is often complicated by the occurrence of neutralizing alloantibodies (inhibitors). Management of patients with inhibitors is challenging and necessitates immune tolerance induction for inhibitor eradication and the use of bypassing agents (activated prothrombin complex concentrates or recombinant activated factor VII), which are expensive and not always effective. Emicizumab is the first humanized bispecific monoclonal therapeutic antibody designed to replace the hemostatic function of activated FVIII by bridging activated factor IX and factor X (FX) to activate FX and allow the coagulation cascade to continue. In the majority of hemophilic patients with and without inhibitors, emicizumab reduced the annualized bleeding rate to almost zero in several clinical trials and demonstrated a good safety profile. However, the concurrent use of emicizumab and activated prothrombin complex concentrate imposes a high risk of thrombotic microangiopathy and thromboembolic events on patients and should be avoided. Yet, the management of breakthrough bleeds and surgery remains challenging with only limited evidence-based recommendations being available. This review summarizes published clinical trials and preliminary reports of emicizumab and discusses the clinical implications of emicizumab in treatment of hemophilia A.

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Section: Bleeding Under Treatment With Emicizumabmentioning

confidence: 99%

Bridging the Missing Link with Emicizumab: A Bispecific Antibody for Treatment of Hemophilia A

et al. 2020

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“…The bispecific antibody and other novel agents should be readily available, when licensed, especially for patients with persistent inhibitors. Clear protocols should be put in place for the management of breakthrough bleeds, as well as for the co-administration of clotting factor concentrates or bypassing agents in case of invasive procedures [12,13].…”

Section: Access To Haemostatic Agentsmentioning

confidence: 99%

European principles of inhibitor management in patients with haemophilia: implications of new treatment options

Hermans

Giangrande

O’Mahony

et al. 2020

Orphanet J Rare Dis

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“…In our opinion, this review article gives us worthy and useful data regarding the safety of planned tooth extractions, which remains the most common surgical procedure due to the prevalence of poor oral health within the population. However, due to novel therapeutical modalities of haemophilia, such as the use of long‐acting factors, gene therapy, substitution and haemostatic rebalancing therapies, the surgical management regimes for invasive procedures in PWH may dramatically change in the very near future . This review importantly signposts areas for important for further research regarding oral surgery in patients with haemophilia to ensure optimal outcomes: Comparison of different haematological treatment protocols, especially protocols with minimal factor replacement support and novel therapies; The influence of severity of the disease, extensiveness of dental surgery, comorbidities, older age and other risk factors on postoperative bleeding; Comparison of efficacy of different local haemostatic agents and measures in prevention of postextraction bleeding in PWH; Dental treatment in patients with inhibitors. …”

Section: Discussionmentioning

confidence: 99%

Current state of play regarding dental extractions in patients with haemophilia: Consensus or evidence‐based practice? A review of the literature

Bajkin

Dougall

2020

Haemophilia

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Due to the global prevalence of oral disease, tooth extraction is the most common surgical procedure required in general population thus likely to be similarly common in patients with haemophilia, especially those in older age and those living in countries with restricted resources. There are little or no consensus about optimal level and duration of factor replacement (FRP) therapy required to prevent bleeding complication following surgery and low levels of evidence to inform protocols and guidelines. The goal of this article was to review the literature regarding haematological treatment protocols and to assess their effectiveness in prevention of bleeding complications during and after tooth extractions in people with haemophilia. A total number of 29 articles were identified. Only two of the studies were randomized controlled trials, and meta‐analysis was not possible. Significant heterogeneity regarding haematological regimes, dental surgical procedures, disease severity and sample size of published studies which are unable to reliably inform the provision of safe dental surgery was noted. Based on the haematological regimens, all studies were classified into one of three groups: pre‐ and postoperative FRP or DDAVP, single preoperative FRP or DDAVP, and no FRP treatment. The overall reported bleeding rate in case of both pre‐ and postoperative FRP and single dose FRP preoperative is similar, 11.9% and 11.4%, respectively, indicating that minimizing the use of clotting factor concentrate is possible if proper local haemostatic measures are provided. Strictly designed prospective studies with higher number of patients are necessary to get firm conclusions about optimal FRP treatment required to prevent bleeding complications during and after oral surgery in patients with haemophilia.

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Cited by 44 publications

References 18 publications

Bridging the Missing Link with Emicizumab: A Bispecific Antibody for Treatment of Hemophilia A

Bridging the Missing Link with Emicizumab: A Bispecific Antibody for Treatment of Hemophilia A

European principles of inhibitor management in patients with haemophilia: implications of new treatment options

Current state of play regarding dental extractions in patients with haemophilia: Consensus or evidence‐based practice? A review of the literature

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