title: 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.
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.
authors: Department of Internal Medicine, Toyonaka Municipal Hospital, Toyonaka, Osaka, Japan.
authors: Takeshi
authors: T
authors: Kotake
authors: Masayoshi
authors: M
authors: Souri
authors: Koji
authors: K
authors: Takada
authors: Satoru
authors: S
authors: Kosugi
authors: Soichi
authors: S
authors: Nakata
authors: Akitada
authors: A
authors: Ichinose
mesh_terms: D000369:Aged, 80 and over; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000627:therapeutic use; D005176:Factor XIII / Q000276:immunology*; D005177:Factor XIII Deficiency / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D006406:Hematoma / Q000150:complications / Q000188:drug therapy / Q000276:immunology; D006470:Hemorrhage / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D012074:Remission Induction; D000069283:Rituximab / Q000627:therapeutic use
chemical_list: D001323:Autoantibodies; D006490:Hemostatics; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide; D005176:Factor XIII