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Semin Thromb Hemost 2022; 48(02): 206-218
DOI: 10.1055/s-0041-1740149
Review Article

A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on Japan

Akitada Ichinose
1   Department of Molecular Pathobiochemistry and Pathobiology, Yamagata University School of Medicine, Yamagata, Japan
2   The Japanese Collaborative Research Group (JCRG) on Autoimmune Acquired Coagulation Factor Deficiencies supported by the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan
,
Tsukasa Osaki
1   Department of Molecular Pathobiochemistry and Pathobiology, Yamagata University School of Medicine, Yamagata, Japan
2   The Japanese Collaborative Research Group (JCRG) on Autoimmune Acquired Coagulation Factor Deficiencies supported by the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan
3   Department of Public Health and Hygiene, Yamagata University Graduate School of Medical Science, Iida-Nishi, Yamagata, Japan
,
Masayoshi Souri
1   Department of Molecular Pathobiochemistry and Pathobiology, Yamagata University School of Medicine, Yamagata, Japan
2   The Japanese Collaborative Research Group (JCRG) on Autoimmune Acquired Coagulation Factor Deficiencies supported by the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan
3   Department of Public Health and Hygiene, Yamagata University Graduate School of Medical Science, Iida-Nishi, Yamagata, Japan
› Author Affiliations Funding This study has been supported by a research aid from Japanese MHLW. The authors are indebted to all members of the Japanese Collaborative Research Group (JCRG) on autoimmune coagulation factor deficiencies (AiCFDs), as well as physicians in charge of acquired factor-V deficiency (AiFVD) cases for their help in collecting patients' clinical information and for participation in the nationwide survey of JCRG.
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Erratum: A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on JapanSemin Thromb Hemost 2022; 48(02): e1-e1
DOI: 10.1055/s-0042-1759569

Abstract

Coagulation factor V (or FV for the purpose of medical safety) is an essential cofactor of coagulation factor X in the common pathway of coagulation; severe FV deficiency leads to a bleeding tendency. Although both congenital and acquired FV deficiencies are widely recognized, FV deficiency also presents as an autoimmune disorder. A nationwide survey on autoimmune coagulation factor deficiencies (AiCFDs) conducted in Japan by our Japanese Collaborative Research Group identified 24 new patients with autoimmune FV deficiency (AiFVD) in the past 5 years. Furthermore, our extensive literature search confirmed that 177 AiFVD cases have been reported in previous articles published from Japan. Patients with AiFVD in Japan were predominantly men, with age similar to those with other AiCFDs. AiFVD was confirmed as a relatively mild type of bleeding diathesis, associated with lower mortality rate than that for AiFVD and other AiCFDs reported in previous studies. Patients with AiFVD had variable FV inhibitor titers and both neutralizing anti-FV autoantibodies and nonneutralizing counterparts. Although spontaneous resolution occurs in some patients, timely initiation of hemostatic and immunosuppressive therapies helps arrest the bleeding and eliminate anti-FV antibodies, resulting in a high cumulative recovery rate. Immunological anti-FV antibody detection is recommended to avoid missing AiFVD cases for the presence of nonneutralizing anti-FV autoantibodies. Further investigation is necessary to clarify the long-term prognosis and optimal management of AiFVD.

Keywords

acquired coagulation factor deficiency - autoantibody - bleeding disorder - factor V - inhibitor - nonneutralizing antibody

Authors' Contributions

A.I. initiated and designed the study, extracted data, wrote, edited, and proofread the manuscript. T.O. conducted experimental examinations, statistical analyses, and proofread the manuscript. M.S. performed experimental examinations and proofread the manuscript.




Publication History

Article published online:
23 December 2021

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