Major surgery management in patients with haemophilia A and inhibitors on emicizumab prophylaxis without global coagulation monitoring

Biron‐Andréani, Christine; Diaz-Cau, Isabelle; Ranc, Alexandre; Navarro, Robert; Léonardi, Christian; Dischino, M; Guy, Romuald; Théron, Alexandre; Garcia-Gournay, Corinne; Santagostino, Elena; Schved, J.F.

doi:10.1111/bjh.16512

Cited by 19 publications

(18 citation statements)

References 11 publications

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“…23,24 The issue of whether, when and for whom emicizumab treatment should be monitored is controversial. 25 However, as our patient population was underrepresented within previous trials, we believed that their longitudinal laboratory monitoring is of special value. As expected, following emicizumab loading, an increase of drug levels was demonstrated, and improved (albeit not normal) TG occurred as well.…”

Section: Discussionmentioning

confidence: 98%

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Emicizumab prophylaxis in haemophilia patients older than 50 years with cardiovascular risk factors: Real‐world data

Misgav

Brutman‐Barazani

Budnik³

et al. 2021

Haemophilia

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Introduction: Emicizumab (Hemlibra™) is approved for prophylaxis of Haemophilia A (HA) patients with and without inhibitors. However, real-world data on emicizumab use in the elderly HA patients with concomitant cardiovascular risk factors are lacking. Aim: To evaluate the safety and efficacy of emicizumab in a real-world cohort of elderly HA patients. Methods: A prospective longitudinal observational study on HA patients over 50 years old treated, followed and monitored during emicizumab prophylaxis was conducted. We documented any bleeding or adverse events and collected plasma samples for emicizumab levels, aPTT and thrombin generation (TG). Results: Seventeen HA patients (2 with inhibitor), whose median age was 62.4 years (range: 51.5-77.1) composed the cohort, including 9/17 with multiple cardiovascular risk factors (high risk group). Seven patients had chronic HIV infection. The median follow-up of our cohort was 400 days (range 89-805, IQR 211-479 days). The median annualized bleeding rate (ABR) significantly decreased for all patients. Among patients who displayed significant bleeding tendencies, emicizumab steady state levels as well as TG were lower as compared with the group. The ABR of four patients concomitantly treated by antiplatelet agents was significantly higher compared with the rest of the cohort. Neither thrombosis nor thrombotic microangiopathy (TMA) was encountered. Conclusions: Emicizumab prophylaxis for HA patients older than 50 years including those with cardiovascular risk factors was well tolerated. As lower emicizumab and TG levels were observed among bleeding patients, we suggest that monitoring laboratory assays could be of value within this age group.

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Section: Discussionmentioning

confidence: 98%

“…The issue of whether, when and for whom emicizumab treatment should be monitored is controversial 25 . However, as our patient population was underrepresented within previous trials, we believed that their longitudinal laboratory monitoring is of special value.…”

Section: Discussionmentioning

confidence: 99%

Emicizumab prophylaxis in haemophilia patients older than 50 years with cardiovascular risk factors: Real‐world data

Misgav

Brutman‐Barazani

Budnik³

et al. 2021

Haemophilia

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“…36 The lack of correlation between TG results and clinical haemostasis in our current cohort may be attributed to the fact that all bleeding episodes of our paediatric patients were trauma-related. The issue of whether, when, and for whom emicizumab treatment should be monitored is controversial, 37 and the current study contributes what we consider to be valuable data to the debate. The role of global assays' monitoring in the surgical settings should be evaluated by prospective future clinical trials.…”

Section: Figmentioning

confidence: 93%

Emicizumab treatment and monitoring in a paediatric cohort: real‐world data

et al. 2020

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Real-world data on emicizumab use and monitoring in paediatric severe haemophilia A (HA) patients are scarce. We therefore sought to evaluate safety, efficacy, and laboratory monitoring of emicizumab prophylaxis in a cohort of 40 children with severe HA, including 22 non-inhibitor patients and nine infants younger than one year. Bleeding, trauma, adverse events, and surgeries were documented during a median follow-up of 45 weeks. Emicizumab levels, activated partial thromboplastin time (aPTT) values, and thrombin generation were measured before and during therapy. Twenty patients experienced zero bleeds. All bleeding was trauma-related, and bleeding risk was positively correlated with the length of emicizumab prophylaxis. Sixteen surgical interventions were performed in 12 patients, with no thrombotic complications or thrombotic microangiopathy. Prolonged aPTT values normalised after emicizumab initiation, correlating with an increase in emicizumab plasma levels. Elevation in the thrombin generation was observed following emicizumab prophylaxis, with lower values recorded in younger infants. Emicizumab prophylaxis was safe and well tolerated. As all bleedings were trauma-related, laboratory monitoring could not predict bleeding risk. Our results do not support routine thrombin generation monitoring in children treated by emicizumab, yet further studies are warranted in the context of surgical procedures.

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“…Finally, it was rapidly observed that maintaining the prophylaxis with emicizumab reduces the duration of post-surgical treatment in major orthopaedic surgeries. 13 Indeed, for our patient, as already reported for adults receiving emicizumab, FVIII or rFVIIa injections were only necessary until the 7th day postsurgery. Thereafter, prevention with emicizumab alone was sufficient to protect against late bleeding during rehabilitation.…”

mentioning

confidence: 57%

“…In pediatrics, only minor procedures were described. 4 5 6 11 12 13 Our case is so, to our knowledge, the first description of a major orthopaedic surgery managed with FVIII concentrates in a child with severe hemophilia A with inhibitor while receiving a prophylaxis with emicizumab. The choice we made of using rFVIII-Fc for bleeding prevention during and after the surgery was foremost driven by the presence of a low titer inhibitor.…”

Section: Figmentioning

confidence: 80%

Management of a High-Risk Surgery with Emicizumab and Factor VIII in a Child with a Severe Hemophilia A and Inhibitor

Lefèvre

Jaffré

Pontis

et al. 2021

TH Open

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Major surgery management in patients with haemophilia A and inhibitors on emicizumab prophylaxis without global coagulation monitoring

Cited by 19 publications

References 11 publications

Emicizumab prophylaxis in haemophilia patients older than 50 years with cardiovascular risk factors: Real‐world data

Emicizumab prophylaxis in haemophilia patients older than 50 years with cardiovascular risk factors: Real‐world data

Emicizumab treatment and monitoring in a paediatric cohort: real‐world data

Management of a High-Risk Surgery with Emicizumab and Factor VIII in a Child with a Severe Hemophilia A and Inhibitor

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