Abstract
Although the incidence of autoimmune hemorrhaphilia due to anti-Factor XIII (FXIII, not FVIII or FXII to avoid confusion) antibodies (AH13) or hemorrhagic “acquired FXIII deficiency due to anti-FXIII autoantibodies” was previously considered rare, it has been on the increase in the twenty-first century, at least in Japan. An 83-year-old woman with an unexplained hemorrhage was admitted to our hospital for intramuscular hematoma and severe anemia. Her FXIII activity was reduced to 10 % of normal; since FXIII inhibitors and anti-FXIII-A subunit autoantibodies were detected, she was definitively diagnosed with AH13. Despite developing cardiac tamponade due to pericardial hemorrhage, she clinically recovered from AH13 after hemostatic therapy with FXIII-concentrates and immunosuppressive treatment with rituximab and cyclophosphamide. However, her FXIII activity remained low and she died of hemorrhage 3.5 years after admission. AH13 patients should be monitored for a prolonged period, as this disease is very likely a chronic intractable hemorrhagic disorder.
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Acknowledgments
This study was supported in part by research aids from the Japanese Ministry of Health, Labor, and Welfare and a research grant from Yamagata University. We would like to thank all members of ‘Japanese Collaborative Research Group on AH13′ and Ms. Yuriko Shibue for their cooperation in conducting a nation-wide survey in Japan from 2009 through 2013. We also thank Dr. Hirokazu Kashiwagi of Osaka University for his helpful discussion and Dr. Kunihiko Kumamoto of Toyonaka-Watanabe Hospital for the patient’s clinical information. A part of this study was reported at the 23rd congress of the International Society of Thrombosis and Hemostasis in July 2011 in Kyoto, Japan.
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Acquired h(a)emophilia is a tentative name for this category of diseases, but unofficial because it is not included in the current version of the WHO ICD-10. “Acquired h(a)emorrhaphilia” seems to be a more logical and proper appellation, because the term hemorrhaphilia stands for “love of bleeding/hemorrhage” while the word hemophilia literally means “love of blood” [Brinkhous, K.M. A short history of hemophilia, with some comments on the word “Hemophilia”. In: KM Brinkhous and HC Hemker (eds). Handbook of Hemophilia, part 1, Amsterdam: Excerpta Medica, American Elsevier. 1975, p. 3–20]. Thus, the authors use the term hemorrhaphilia for a bleeding disorder caused by anti-FXIII autoantibodies, throughout this manuscript. H(a)emophilia must be used for the inherited hemorrhagic disease due to Factor VIII deficiency as listed in WHO ICD-10.
‘Clinical’ remission is defined as the disappearance of all bleeding symptoms, in this manuscript.
Masayoshi Souri and Akitada Ichinose belong to Japanese Collaborative Research Group on AH13.
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Kotake, T., Souri, M., Takada, K. et al. Report of a patient with chronic intractable autoimmune hemorrhaphilia due to anti-factor XIII/13 antibodies who died of hemorrhage after sustained clinical remission for 3 years. Int J Hematol 101, 598–602 (2015). https://doi.org/10.1007/s12185-015-1754-8
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DOI: https://doi.org/10.1007/s12185-015-1754-8