Abstract
Autoimmune hemorrhaphilia due to anti-factor XIII (FXIII) antibodies (AH13) is a life-threatening disease associated with high risk of surgical bleeding. Since AH13 occurs mainly in the elderly, patients of AH13 tend to be complicated with other life-threatening diseases that may require surgical procedures. During our nation-wide survey on AH13, supported by the Japanese Ministry of Health, Labor, and Welfare, patients with unexplained bleeding were examined for FXIII-related parameters and anti-FXIII autoantibodies. A 64-year-old man had previously been tentatively diagnosed with AH13 and received immunosuppressive therapies, as FXIII inhibitor was detected by functional cross-mixing studies. About 2 years later, he was definitively diagnosed with AH13, because our immuno-chromatographic test and enzyme-linked immuno-sorbent assay detected FXIII-bound anti-FXIII-A subunit autoantibodies. Since routine endoscopic examination revealed suspected esophageal carcinoma, a preparatory FXIII pharmacokinetic (PK) analysis was performed by infusing FXIII concentrates prior to biopsy. Consequently, biopsy of this lesion was done without bleeding complications. One month later, a second PK study was carried out before surgery, and esophageal bypass surgery was completed successfully under FXIII replacement therapy. Our experience with this case suggests that operations can be performed safely and with confidence even in patients with such life-threatening hemorrhagic diseases.
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Acknowledgments
This study was approved by the institutional review board of Yamagata University School of Medicine. All procedures were conducted in accordance with the Declaration of Helsinki. Written informed consent was obtained from this patient. 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 (JCRG) on AH13’ and Ms. Yuriko Shibue for their cooperation in conducting a nation-wide survey in Japan from 2009 through 2014. The authors also thank Drs. Koji Okamoto, Kento Sakon and Masahito Yano for their invaluable advices on surgical problems of the present case.
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Kojima, M., Ichinose, A., Souri, M. et al. Successful bypass surgery for esophageal carcinoma under adequate factor XIII/13 replacement therapy in a case of intractable autoimmune hemorrhaphilia due to anti-Factor XIII/13 antibodies. Int J Hematol 103, 341–347 (2016). https://doi.org/10.1007/s12185-015-1917-7
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DOI: https://doi.org/10.1007/s12185-015-1917-7