Challenges of biological monitoring in a hemophilia A patient without inhibitors on emicizumab undergoing major orthopedic surgery: a case report

Guillaume, Louise; Dievoet, Marie-Astrid van; Lambert, Catherine; Hermans, Cédric

doi:10.1177/20406207211040345

Cited by 5 publications

(9 citation statements)

References 41 publications

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“…However, in PWHA without inhibitors, the rate of thrombotic events (including TMA) in association with emicizumab and FVIII is negligible, 132 because the combination of emicizumab and FVIII does not appear to enhance hemostatic activity. 133 Recently, an overview of surgical procedures from the HAVEN 1-4 trials has been reported. 134 In this overview, thromboprophylaxis was not mentioned.…”

Section: Clinical Question 12: Can Systemic Thrombolysis Be Given In ...mentioning

confidence: 99%

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Antithrombotic Treatment in Patients With Hemophilia: an EHA-ISTH-EAHAD-ESO Clinical Practice Guidance

et al. 2023

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Cardiovascular disease is an emerging medical issue in patients with hemophilia (PWH) and its prevalence is increasing up to 15% in PWH in the United States. Atrial fibrillation, acute and chronic coronary syndromes, venous thromboembolism, and cerebral thrombosis are frequent thrombotic or prothrombotic situations, which require a careful approach to fine-tune the delicate balance between thrombosis and hemostasis in PWH when using both procoagulant and anticoagulant treatments. Generally, PWH could be considered as being naturally anticoagulated when clotting factors are <20 IU/dL, but specific recommendations in patients with very low levels according to the different clinical situations are lacking and mainly based on the anecdotal series. For PWH with baseline clotting factor levels >20 IU/dL in need for any form of antithrombotic therapy, usually treatment without additional clotting factor prophylaxis could be used, but careful monitoring for bleeding is recommended. For antiplatelet treatment, this threshold could be lower with single-antiplatelet agent, but again factor level should be at least 20 IU/dL for dual antiplatelet treatment. In this complex growing scenario, the European Hematology Association in collaboration with the International Society on Thrombosis and Haemostasis, the European Association for Hemophilia and Allied Disorders, the European Stroke Organization, and a representative of the European Society of Cardiology Working Group on Thrombosis has produced this current guidance document to provide clinical practice recommendations for health care providers who care for PWH.

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Section: Clinical Question 12: Can Systemic Thrombolysis Be Given In ...mentioning

confidence: 99%

Section: Section 5: Venous Thromboembolismmentioning

confidence: 99%

Antithrombotic Treatment in Patients With Hemophilia: an EHA-ISTH-EAHAD-ESO Clinical Practice Guidance

et al. 2023

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“…Surgical experience in patients on emicizumab out of clinical trials is being increasingly reported in form of either case reports 34,37–39,41,43–46,64–65 or case series 33,40,42,47 . Table 2 summarizes the real‐world experience reported so far in form of case reports.…”

Section: Invasive Procedures In the Era Of Non‐factor Replacement The...mentioning

confidence: 99%

“…All procedures were managed with rFVIIa +/tranexamic acid without any thrombotic complication. 32 Surgical experience in patients on emicizumab out of clinical trials is being increasingly reported in form of either case reports 34,[37][38][39]41,[43][44][45][46][64][65] or case series. 33,40,42,47 Table 2 summarizes the real-world experience reported so far in form of case reports.…”

Section: Perioperative Management Of Pwh Treated With Emicizumabmentioning

confidence: 99%

“…To date, limited information on surgical management of PWH on prophylaxis with fitusiran and anti‐TFPI is available from clinical trials 27–29 . On the other hand, more data have been collected and published on the surgical management of patients on emicizumab prophylaxis both during the clinical trial program 30–32 and in the real‐world setting 33–47 …”

Section: Introductionmentioning

confidence: 99%

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Managing invasive procedures in haemophilia patients with limited resources, extended half‐life concentrates or non‐replacement therapies in 2022

2022

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New treatment possibilities and modalities are now available globally for patients with haemophilia requiring surgery or invasive procedures. The first is the appropriate application of low‐dose protocols of clotting factor concentrates (CFC) achieving adequate perioperative haemostasis in resources constraint environments. The increasing availability of CFC through humanitarian aid programs allows more invasive surgeries to be performed for which efficacy and safety data should be more widely collected and reported. Second, extended half‐life CFC that are increasingly available in many countries represent valuable alternatives to standard half‐life products in surgical patients allowing reduced number of infusions and lower consumption, in particular for extended half‐life factor IX. Third, in the era of recently introduced nonfactor prophylaxis, some minor surgical procedures can now be performed without additional haemostatic treatment, others with few low‐dose administrations of CFC or bypassing agents. Additional factor VIII/IX or recombinant activated factor VII has proven to be safe and effective in association with emicizumab for major surgeries and it was effectively given at low doses in association with fitusiran (including activated prothrombin complex concentrate). No thrombotic complications have been reported in the surgical setting so far. A multidisciplinary team/facility remains crucial to manage major surgery in patients on prophylaxis with these new agents.

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Thrombin Generation Assay to Support Hematologists in the Era of New Hemophilia Therapies

Josset,

Rezigue,

Dargaud

2024

Int J Lab Hematology

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Hematology laboratories have traditionally monitored hemophilia replacement therapy by measuring coagulation factors before and after infusion. However, new drugs that do not rely on the replacement of the deficient factor require new approaches to laboratory monitoring, as factor VIII (FVIII) or factor IX (FIX) assays are no longer adequate. Non‐factor therapies come in many different forms, that have one thing in common: they all increase thrombin generation. Their main adverse effect is thrombosis which may occur when too much thrombin is formed. This is the perfect mirror image of anticoagulant treatment, which always diminishes the amount of thrombin formed and has bleeding as its main adverse effect. Thrombin‐forming capacity is decreased in congenital bleeding disorders and increased in prothrombotic conditions, indicating it governs bleeding and thrombosis. Therefore, the thrombin generation assay (TGA) is a logical tool for monitoring non‐factor therapies, offering a comprehensive assessment of hemostatic balance. TGA identifies patients with severe bleeding, helps to optimize bypassing therapy, and detects hypercoagulability, making it ideal for guiding and monitoring hemophilia treatment with non‐factor therapies. It also assesses the efficacy and safety of combined therapies, including non‐factor therapies with bypassing agents or FVIII/FIX concentrates. The purpose of this paper is to review the current state of knowledge regarding the use of TGA to monitor novel hemophilia therapies. It will address controversies, limitations, and knowledge gaps related to the integration of TGA into personalized medicine in routine clinical practice.

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Challenges of biological monitoring in a hemophilia A patient without inhibitors on emicizumab undergoing major orthopedic surgery: a case report

Cited by 5 publications

References 41 publications

Antithrombotic Treatment in Patients With Hemophilia: an EHA-ISTH-EAHAD-ESO Clinical Practice Guidance

Antithrombotic Treatment in Patients With Hemophilia: an EHA-ISTH-EAHAD-ESO Clinical Practice Guidance

Managing invasive procedures in haemophilia patients with limited resources, extended half‐life concentrates or non‐replacement therapies in 2022

Thrombin Generation Assay to Support Hematologists in the Era of New Hemophilia Therapies

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