The first reported case of acquired haemophilia A in which bleeding episodes were successfully treated via administration of a single‐dose mixture of activated factor VIIa/X

Yamada, Shinya; Arahata, Masahisa; Morishita, Eriko; Ichinose, Akitada; Asakura, Hitoshi

doi:10.1111/hae.13843

Cited by 4 publications

(4 citation statements)

References 8 publications

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“…Pd-FVIIa/FX, a 1 : 10 ratio mixture of FVIIa and its substrate FX, is a new BPA. Although its safety and efficacy has been reported for patients with hemophilia A with inhibitors [8], there is only one case report of its use for AHA [7]. The drug has a stronger hemostatic ability than FVIIa alone in a monkey hemophilia B model and less thrombogenicity than APCC in the rabbit venous stasis model [9].…”

Section: Discussionmentioning

confidence: 99%

“…The drug has since been used in combination with conventional BPAs and shown to be effective in a small number of perioperative patients with hemophilia A with inhibitors [6]. In addition, it has been used as monotherapy in an AHA patient with bleeding [7]. However, there are no reports on combining pd-FVIIa/ FX with a conventional BPA in an AHA patient with refractory bleeding.…”

Section: Introductionmentioning

confidence: 99%

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The safety of the combination therapy of recombinant factor VIIa and plasma-derived factor VIIa and factor X for refractory hemorrhage in acquired hemophilia A

Okayama

Bingo

Sakatoku

et al. 2023

Blood Coagulation &Amp; Fibrinolysis

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Acquired hemophilia A (AHA) is a rare, life-threatening hemorrhagic disease caused by autoantibodies against factor VIII (FVIII), and bypassing agents (BPA) are used to control bleeding. However, some cases need a change of BPA or BPAs given sequentially or in combination for refractory bleeding. A 71-year-old man was admitted with subcutaneous hemorrhage. Laboratory investigations showed prolongation of activated partial thromboplastin time (APTT) and low-coagulation FVIII activity and FVIII inhibitor; we, therefore, diagnosed AHA. He was treated with recombinant factor VIIa (rFVIIa) BPA and prednisolone. However, his symptoms did not improve sufficiently, thus we switched BPA to activated prothrombin complex concentrate. Unfortunately, this was not effective and he suffered hemorrhagic shock. Therefore, we selected rFVIIa, with plasma-derived FVIIa and factor X (pd-FVIIa/FX) as combination therapy, and hemostasis was achieved without thrombosis. This case suggests that the combination of rFVIIa and pd-FVIIa/FX short-term can be well tolerated for refractory hemorrhage in AHA.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The safety of the combination therapy of recombinant factor VIIa and plasma-derived factor VIIa and factor X for refractory hemorrhage in acquired hemophilia A

Okayama

Bingo

Sakatoku

et al. 2023

Blood Coagulation &Amp; Fibrinolysis

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“…15 rFVIIa was administered in 13% of cases. FX, which is downstream to FVII in the coagulation cascade, is drastically reduced; therefore, the use of recently marketed plasma-derived FX/FVIIa concentrates is reasonable, 50,51,54 although not covered by insurance.…”

Section: Hemostatic Therapymentioning

confidence: 99%

“…21 It is also because the administered FVIII preparations are easily overwhelmed by high-titer anti-FVIII autoantibodies, and hemostatic effects are rarely observed. Recently, plasma-derived FX/FVIIa 50 51 and a so-called bispecific antibody preparation (Emicizumab; Hemlibra, Chugai Pharmaceutical Co., Tokyo, Japan) 52 have been marketed and covered by health insurance for AiF8D.…”

Section: Treatment and Prognosismentioning

confidence: 99%

Diagnosis and Treatment of Autoimmune Acquired Coagulation Factor Deficiencies: An Evidence-Based Review of Japanese Practice

Ichinose,

Osaki,

Souri

2024

Semin Thromb Hemost

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Among the acquired coagulation factor deficiencies, autoimmune coagulation factor deficiencies (AiCFD) are rare and result from autoantibody production against coagulation factors. In Japan, a nationwide survey on AiCFD has been conducted since 2009. Autoimmune factor XIII, factor VIII, von Willebrand factor, factor V, and factor X deficiencies (AiF13D, AiF8D, AiVWFD, AiF5D, and AiF10D, respectively) have been enacted as “designated intractable disease-282.” The incidence of AiF8D, AiF13D, and AiF5D was 1.83, 0.044, and 0.038 per million people/year, respectively, whereas that of AiVWFD and AiF10D was not calculable owing to the small number of patients. AiF13D and AiF8D were often idiopathic, whereas AiVWFD was often associated with plasma cell neoplasms. Epistaxis was a characteristic symptom of AiVWFD, intramuscular bleeding was frequent in AiF13D and AiF8D, and subcutaneous bleeding (purpura) was frequent in AiF13D and AiF10D, although none were specific to any one disease. Differential diagnosis cannot be made based on bleeding symptoms alone; therefore, rapid and accurate testing is mandatory. Definitive diagnosis of AiCFD necessitates identifying the presence of coagulation factor “inhibitors” and/or “autoantibodies.” Therefore, these tests should be performed upon unexplained severe acquired coagulation factor deficiencies. The mainstay of treatment for AiCFD was hemostatic therapy and autoantibody eradication therapy, which included the replacement of coagulation factors or “bypass” agents and administration of immunosuppressants. The rate of hemorrhagic death was high in AiF13D (13%), followed by AiF5D (7%) and Ai10D (5%); therefore, early diagnosis and optimal treatment are essential for AiCFDs. Given the unknown long-term prognosis, “intractable disease platform registries” have begun to accumulate in Japan.

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Effects of low and high factor X concentrations on thrombin generation in vitro

et al. 2022

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The first reported case of acquired haemophilia A in which bleeding episodes were successfully treated via administration of a single‐dose mixture of activated factor VIIa/X

Cited by 4 publications

References 8 publications

The safety of the combination therapy of recombinant factor VIIa and plasma-derived factor VIIa and factor X for refractory hemorrhage in acquired hemophilia A

The safety of the combination therapy of recombinant factor VIIa and plasma-derived factor VIIa and factor X for refractory hemorrhage in acquired hemophilia A

Diagnosis and Treatment of Autoimmune Acquired Coagulation Factor Deficiencies: An Evidence-Based Review of Japanese Practice

Effects of low and high factor X concentrations on thrombin generation in vitro

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