title: Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan.
abstract: Acquired hemophilia A (AHA), which is caused by autoantibodies against coagulation factor VIII (FVIII) is a rare, life-threatening bleeding disorder, the incidence of which appears to be increasing in Japan as the population ages. However, the clinical characteristics, treatment, and outcomes of AHA remain difficult to establish due to the rarity of this disease. We retrospectively analyzed data from 25 patients (median age 73 years; range 24-92 years; male n = 15) diagnosed with AHA between 1999 and 2015 at Gunma University Hospital. We identified autoimmune diseases and malignancy as underlying conditions in four and three patients, respectively. Factor VIII activity was significantly decreased in all patients (median 2.0%; range <1.0-8.0) by FVIII inhibitor (median 47.0 BU/mL; range 2.0-1010). Among 71 bleeding events, subcutaneous or intramuscular hemorrhage was the most prevalent. Seventeen patients required bypassing agents. Twenty-two (91.7%) of 24 patients treated with immunosuppressive agents achieved complete response (CR) during a median of 57.5 days (range 19-714 days). Although three patients (12%) relapsed and seven (28%) died of infection, none of the deaths were related to bleeding. Although most of our patients achieved CR after immunosuppressive therapy, the rate of infection-related mortality was unsatisfactorily high.
authors.affiliation: Department of Medicine and Clinical Sciences, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan. yo-ogawa@gunma-u.ac.jp.|The Japanese Collaborative Research Group (JCRG) on Acquired Coagulopathies Supported by the Japanese Ministry of Health, Labour and Welfare, Yamagata, Japan. yo-ogawa@gunma-u.ac.jp.
authors.forename: Yoshiyuki
authors.identifier: http://orcid.org/0000-0003-1459-3970
authors.initials: Y
authors.lastname: Ogawa
authors.affiliation: Department of Medicine and Clinical Sciences, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Kunio
authors.initials: K
authors.lastname: Yanagisawa
authors.affiliation: Department of Medicine and Clinical Sciences, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Uchiumi
authors.affiliation: Department of Medicine and Clinical Sciences, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Takuma
authors.initials: T
authors.lastname: Ishizaki
authors.affiliation: Department of Medicine and Clinical Sciences, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Takeki
authors.initials: T
authors.lastname: Mitsui
authors.affiliation: Department of Internal Medicine, National Hospital Organization Takasaki General Medical Center, Takasaki, Japan.
authors.forename: Fumito
authors.initials: F
authors.lastname: Gouda
authors.affiliation: The Japanese Collaborative Research Group (JCRG) on Acquired Coagulopathies Supported by the Japanese Ministry of Health, Labour and Welfare, Yamagata, Japan.|Department of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido, Sapporo, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ieko
authors.affiliation: The Japanese Collaborative Research Group (JCRG) on Acquired Coagulopathies Supported by the Japanese Ministry of Health, Labour and Welfare, Yamagata, Japan.|Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan.
authors.forename: Akitada
authors.initials: A
authors.lastname: Ichinose
authors.affiliation: Renal Division, Japan Red Cross Maebashi Hospital, Maebashi, Japan.
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nojima
authors.affiliation: Department of Medicine and Clinical Sciences, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Handa
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D001780:Blood Coagulation Tests; D015897:Comorbidity; D019468:Disease Management; D005169:Factor VIII / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007564:Japan; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012008:Recurrence; D012189:Retrospective Studies; D000069283:Rituximab / Q000008:administration & dosage / Q000627:therapeutic use; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D007166:Immunosuppressive Agents; D000069283:Rituximab; C078147:F8 protein, human; D005169:Factor VIII
keywords: Acquired hemophilia A; Autoimmune disease; Coagulation factor VIII; Immunosuppressive therapy; Rituximab