title: Autoimmune Hemorrhaphilia Resulting from Autoantibody against the A Subunit of Factor XIII.
abstract: A 65-year-old woman was admitted with acute intramuscular hemorrhage of the left gluteus medius and piriformis muscles and associated anemia. Blood tests showed low plasma factor XIII (FXIII) antigen and activity. A cross-mixing test revealed a concave "inhibitor" pattern and anti-FXIII-A subunit antibody was detected. The patient was diagnosed with autoimmune hemorrhaphilia resulting from anti-FXIII antibody. The bleeding has not recurred since the initiation of treatment with oral immunosuppressive agents. Although hemorrhagic acquired FXIII deficiency is a rare disorder, prompt recognition of the underlying mechanism can save lives.
authors.affiliation: Department of Hematology, Tokyo Medical and Dental University, Japan.
authors.forename: Emi
authors.initials: E
authors.lastname: Uchida
authors.forename: Ken
authors.initials: K
authors.lastname: Watanabe
authors.forename: Reina
authors.initials: R
authors.lastname: Arai
authors.forename: Masahide
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Souri
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Osaki
authors.forename: Akitada
authors.initials: A
authors.lastname: Ichinose
authors.forename: Osamu
authors.initials: O
authors.lastname: Miura
authors.forename: Takatoshi
authors.initials: T
authors.lastname: Koyama
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D005176:Factor XIII / Q000276:immunology*; D005177:Factor XIII Deficiency / Q000209:etiology*; D005260:Female; D006470:Hemorrhage / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018482:Muscle, Skeletal
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D005176:Factor XIII