title: [Cyclosporine A as an effective treatment for a patient with acquired hemophilia A complicated with diabetes mellitus and ischemic heart disease].
abstract: Acquired hemophilia A (AHA) is a rare coagulation disorder due to the development of an autoantibody against and inhibitor of coagulation factor VIII. It has been reported that immunosuppressive therapy with corticosteroids, cyclophosphamide, azathioprine and vincristine are effective to decrease this inhibitor. When corticosteroids and cytotoxic drugs are ineffective, cyclosporine A (CyA) may be effective as a second-line salvage therapy. Except for postpartum conditions, AHA usually occurs in elderly patients who are often already suffering from diabetes mellitus, ischemic heart disease and/or hyperlipidemia. However, immunosuppressive and cytotoxic drugs may have adverse effects on these patients. We report on a 66-year-old man who developed AHA after colon cancer resection (factor VIII inhibitor: 61 Bethesda units/ml, aPTT : 97.9 s). Since he already had both diabetes mellitus and ischemic heart disease, we abandoned treatment with corticosteroids and oral cyclophosphamide was started, but was switched to CyA because of leukopenia. Within 3 months of starting the CyA treatment, aPTT levels returned to normal and 4 further months were required for complete eradication of the inhibitor. This case revealed that CyA is as effective as corticosteroids for AHA. For patients with AHA who have unfavorable complications due to corticosteroids and cytotoxic drugs, CyA could be a potential first-line drug.
authors.affiliation: Hematology Department, Iwate Prefectural Central Hospital.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Wano
authors.forename: Yookija
authors.initials: Y
authors.lastname: Kang
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Masaki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawabata
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Fukushima
authors.forename: Kumiko
authors.initials: K
authors.lastname: Shimoyama
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Satoh
authors.forename: Yasuro
authors.initials: Y
authors.lastname: Miyairi
authors.forename: Mitsutoshi
authors.initials: M
authors.lastname: Kashiwaba
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hirose
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D016572:Cyclosporine / Q000627:therapeutic use*; D048909:Diabetes Complications; D005169:Factor VIII / Q000276:immunology; D006467:Hemophilia A / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D017202:Myocardial Ischemia / Q000150:complications*; D016879:Salvage Therapy; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D005169:Factor VIII