Measurement of coagulation factor antibody levels is useful for diagnosis and determining therapeutic efficacy in hemorrhagic patients with autoantibodies to coagulation factor VIII and factor V: results from a single center in Japan

Ieko, Masahiro; Ohmura, Koichiro; Naito, Sumiyoshi; Yoshida, Mika; Saito, Makoto; Kiyohara, Kazuki; Miyazima, Shinri; Maeta, Takahiro; Ohtsu, Akihiro; Shimosegawa, Kenji; Takahashi, Nobuhiko; Ichinose, Akitada

doi:10.1007/s12185-021-03212-9

Cited by 7 publications

(4 citation statements)

References 32 publications

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“…Several cases of AiFVD co-detected with LA have been reported [5,43,[45][46][47][48][49][50][51]. The confirmed cases of AiFVD co-detected with LA presented in the literature are shown in Table 2.…”

Section: Aifvd Cases With Lamentioning

confidence: 84%

“…Most clinical manifestations observed in AiFVD cases are associated with bleeding, although reports have noted that some have been related to thrombotic manifestations [41,42]. Notably, FV not only plays a coenzyme-like role in promoting coagulation but also participates in anticoagulation by protein C [43]. Bleeding symptoms in individuals with AiFVD have been reported to be relatively mild compared with the symptoms in those with AiCFD, although some patients with AiFVD may show spontaneous remission [3].…”

Section: Aifvdmentioning

confidence: 99%

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Considerations for simultaneous detection of autoantibodies to coagulation factor and lupus anticoagulant

Ieko,

Ohmura,

Naito

et al. 2023

Explor Immunol

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In patients with autoimmune coagulation factor deficiency (AiCFD), the production of autoantibodies that inhibit coagulation factors in the blood reduces the activity of those relevant coagulation factors, resulting in severe bleeding symptoms. Recently, reports of patients with AiCFD have noted the concomitant detection of lupus anticoagulant (LA), a risk factor for thrombosis. LA-positive patients may show bleeding symptoms due to decreased activity of coagulation factor II (FII) caused by autoantibodies against FII, in addition to thrombotic symptoms, a condition termed LA-hypoprothrombinemia syndrome (LAHPS). Anti-FII antibodies in LAHPS cases are frequently cleared antibodies that can be detected using immunological techniques, such as enzyme-linked immunosorbent assay (ELISA). Recently, several cases of coagulation FV inhibitors, known as autoimmune FV deficiency, have been reported. Some of these cases may be complicated by LA, which can cause thrombosis. False-positive results for anticoagulant inhibitors are known to occur in LA cases; therefore, immunological confirmation of antibodies against coagulation factors is recommended. Additionally, acquired hemophilia A (AHA), caused by autoantibodies against FVIII, is a typical acquired hemorrhagic diathesis, although affected patients may present with thrombosis associated with LA. Thus, it is important to remember that hemorrhagic diathesis due to autoantibodies against clotting factors can also result in thrombosis, as demonstrated by the co-detection of LA. When clotting factor inhibitors are detected in LA-positive individuals, it is important to confirm the presence of autoantibodies against coagulation factors using immunological methods, such as ELISA, to avoid false-positive results.

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Section: Aifvd Cases With Lamentioning

confidence: 84%

Section: Aifvdmentioning

confidence: 99%

Considerations for simultaneous detection of autoantibodies to coagulation factor and lupus anticoagulant

Ieko,

Ohmura,

Naito

et al. 2023

Explor Immunol

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“…Presently, anti-FVIII and anti-FV antibody detection kits (Zymutest Anti-FVIII IgG Mono Strip and Anti-FV IgG detection kit; both from Hyphen BioMed, Neuville sur Oise, France) are commercially available 33,63 in Japan (and in Europe); however, commercial anti-FXIII, anti-VWF, and anti-FX antibody detection kits are not. We have developed and implemented homemade detection tests for these diseases.…”

Section: Autoantibody Detection Methodsmentioning

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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“…Anti-FVAb could not be detected using functional coagulation activity tests; therefore, immunological tests should be used to detect antibodies in cases of suspected FV inhibitor-negative AiFVD. 2,5,9 AiFVD is frequently associated with other autoimmune diseases, neoplastic diseases, pregnancy and delivery, antibiotics, and antipsychotic medications; however, approximately half of the cases are idiopathic. 2 In this case, no underlying disease was found to be a clear cause of AiFVD.…”

mentioning

confidence: 99%

Autoimmune‐acquired coagulation factor V deficiency with hyperfibrinolytic disseminated intravascular coagulation

Kaneda,

Fukuno,

Minami

et al. 2023

Int J Lab Hematology

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Measurement of coagulation factor antibody levels is useful for diagnosis and determining therapeutic efficacy in hemorrhagic patients with autoantibodies to coagulation factor VIII and factor V: results from a single center in Japan

Cited by 7 publications

References 32 publications

Considerations for simultaneous detection of autoantibodies to coagulation factor and lupus anticoagulant

Considerations for simultaneous detection of autoantibodies to coagulation factor and lupus anticoagulant

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

Autoimmune‐acquired coagulation factor V deficiency with hyperfibrinolytic disseminated intravascular coagulation

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