title: Aggressive fatal case of autoimmune hemorrhaphilia resulting from anti-Factor XIII antibodies.
abstract: Factor XIII (FXIII) is a fibrin-stabilizing factor consisting of catalytic A subunits (FXIII-A) and carrier B subunits (FXIII-B). Congenital FXIII deficiency is a rare bleeding disorder. Acquired FXIII deficiency resulting from FXIII hypo-synthesis and/or hyperconsumption is a relatively common disorder in which patients seldom bleed. On the contrary, 'autoimmune/acquired hemorrhaphilia XIII/13 due to anti-FXIII antibodies (AH13)' is a rare but life-threatening bleeding disorder. Through a nationwide survey of AH13, we diagnosed aggressive AH13 in a 66-year-old woman. She consulted our department because of a spontaneous hematoma in her hand. After 1.5 months, she also developed an intramuscular hematoma but retained approximately half (52%) of the normal FXIII activities. The patient's bleeding symptoms were aggravated to catastrophic massive bleedings in the large abdominal muscles and intrapelvic and intraperitoneal spaces. Two months after the bleeding onset, she died despite undergoing plasma exchange, which was performed because we were deeply suspicious of the presence of an anti-FXIII inhibitor. Seven days after her death, extremely low FXIII activity (6%) and positive data on anti-FXIII inhibitor were reported by a commercial laboratory. Our dot blot assay detected anti-FXIII-A autoantibodies, afterwards. Thus, the diagnosis of aggressive AH13 as early as possible is necessary to save patients' lives.
authors.affiliation: Department of Hematology, Saiseikai Noe Hospital, 1-3-25 Furuichi, Joto-ku, Osaka, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Sugiyama
authors.forename: Hiroko
authors.initials: H
authors.lastname: Uesugi
authors.forename: Satoshi
authors.initials: S
authors.lastname: Suzuki
authors.forename: Kenji
authors.initials: K
authors.lastname: Tanaka
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Souri
authors.forename: Akitada
authors.initials: A
authors.lastname: Ichinose
mesh_terms: D000368:Aged; D000900:Anti-Bacterial Agents / Q000009:adverse effects / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology* / Q000628:therapy; D002443:Ceftriaxone / Q000009:adverse effects / Q000627:therapeutic use; D057210:Delayed Diagnosis; D018450:Disease Progression; D004844:Epistaxis / Q000209:etiology; D005176:Factor XIII / Q000037:antagonists & inhibitors / Q000276:immunology*; D005177:Factor XIII Deficiency / Q000097:blood / Q000276:immunology* / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D005884:Gingival Hemorrhage / Q000209:etiology; D006406:Hematoma / Q000209:etiology; D017583:Hemodiafiltration; D006465:Hemoperitoneum / Q000209:etiology; D006474:Hemorrhagic Disorders / Q000097:blood / Q000276:immunology*; D006801:Humans; D009494:Neurosyphilis / Q000150:complications / Q000188:drug therapy; D010314:Partial Thromboplastin Time; D010951:Plasma Exchange
chemical_list: D000900:Anti-Bacterial Agents; D001323:Autoantibodies; D002443:Ceftriaxone; D005176:Factor XIII