title: 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.
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.
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Minoru
authors.initials: M
authors.lastname: Kojima
authors.affiliation: Department of Molecular Patho-Biochemistry and -Biology, Yamagata University School of Medicine, Yamagata, Japan. aichinos@med.id.yamagata-u.ac.jp.|Japanese Collaborative Research Group on AHXIII/13 (Japanese Ministry of HLW), Yamagata, Japan. aichinos@med.id.yamagata-u.ac.jp.
authors.forename: Akitada
authors.initials: A
authors.lastname: Ichinose
authors.affiliation: Department of Molecular Patho-Biochemistry and -Biology, Yamagata University School of Medicine, Yamagata, Japan.|Japanese Collaborative Research Group on AHXIII/13 (Japanese Ministry of HLW), Yamagata, Japan.
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Souri
authors.affiliation: Department of Molecular Patho-Biochemistry and -Biology, Yamagata University School of Medicine, Yamagata, Japan.|Japanese Collaborative Research Group on AHXIII/13 (Japanese Ministry of HLW), Yamagata, Japan.
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Osaki
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Hidetsugu
authors.initials: H
authors.lastname: Kawai
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Amaki
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Numata
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Mitsuki
authors.initials: M
authors.lastname: Miyamoto
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Ogiya
authors.affiliation: Department of Internal Medicine, Nagaoka Chuo General Hospital, Nagaoka, Japan.
authors.forename: Kosuke
authors.initials: K
authors.lastname: Tsuboi
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Ogawa
authors.affiliation: Department of Gastroenterological Surgery, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Soji
authors.initials: S
authors.lastname: Ozawa
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan.
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Ando
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000276:immunology*; D002277:Carcinoma / Q000601:surgery*; D013505:Digestive System Surgical Procedures / Q000379:methods; D004938:Esophageal Neoplasms / Q000601:surgery*; D005176:Factor XIII / Q000008:administration & dosage* / Q000276:immunology* / Q000493:pharmacokinetics; D006467:Hemophilia A / Q000209:etiology* / Q000276:immunology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011300:Preoperative Care; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D005176:Factor XIII
keywords: Anti-FXIII/13 antibodies; Autoimmune/acquired hemorrhaphilia; Factor XIII concentrates; Immunosuppressive therapy; Pharmacokinetic study