title: Successful Management of a Patient with Autoimmune Hemorrhaphilia due to Anti-Factor XIII/13 Antibodies Complicated by Pulmonary Thromboembolism.
abstract: Autoimmune hemophilia-like disease (hemorrhaphilia) due to anti-factor XIII (FXIII) antibodies (AH13) is a very rare, life-threatening bleeding disorder. A 77-year-old woman developed macrohematuria and a right renal pelvic hematoma. The coagulation times were not prolonged, but FXIII activity and antigen levels were severely and moderately reduced to 9 and 29% of normal values, respectively. Accordingly, the FXIII-specific activity turned out to be low. FXIII inhibitor and anti-FXIII-A subunit autoantibodies were detected by a 1:1 crossmixing test and immunoblot and immunochromatographic assays. She was therefore diagnosed with "definite AH13" and treated with plasma-derived FXIII concentrates to arrest the hemorrhage. In addition to a highly compressed inferior vena cava by a huge renal pelvic hematoma, deep vein thrombosis (DVT) and pulmonary thromboembolism (PE) were identified by systemic computed tomography. The patient was immediately started on anticoagulation therapy with low-dose heparin. Emboli disappeared quickly, probably because under-crosslinked thrombi caused by severe FXIII deficiency are vulnerable to fibrinolysis. After about 1.5 years, anti-FXIII-A subunit autoantibodies still remained despite the use of rituximab, steroid pulse therapy, oral prednisolone, and oral cyclophosphamide treatments. In conclusion, an extremely rare AH13 case complicated by DVT and PE was successfully managed by balancing anticoagulation therapy with hemostatic therapy.
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, Maebashi, Japan.
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ogawa
authors.forename: Kunio
authors.initials: K
authors.lastname: Yanagisawa
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Souri
authors.forename: Masahiro
authors.initials: M
authors.lastname: Mihara
authors.forename: Chiaki
authors.initials: C
authors.lastname: Naito
authors.forename: Makiko
authors.initials: M
authors.lastname: Takizawa
authors.forename: Takuma
authors.initials: T
authors.lastname: Ishizaki
authors.forename: Takeki
authors.initials: T
authors.lastname: Mitsui
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Handa
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Osaki
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nojima
authors.forename: Akitada
authors.initials: A
authors.lastname: Ichinose
mesh_terms: D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology / Q000628:therapy*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005176:Factor XIII / Q000037:antagonists & inhibitors* / Q000276:immunology* / Q000627:therapeutic use; D005177:Factor XIII Deficiency / Q000150:complications* / Q000276:immunology / Q000628:therapy*; D005260:Female; D006406:Hematoma / Q000150:complications / Q000000981:diagnostic imaging; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007674:Kidney Diseases / Q000150:complications / Q000000981:diagnostic imaging; D011655:Pulmonary Embolism / Q000150:complications*; D000069283:Rituximab / Q000627:therapeutic use; D020246:Venous Thrombosis / Q000150:complications
chemical_list: D000925:Anticoagulants; D001323:Autoantibodies; D000069283:Rituximab; D003520:Cyclophosphamide; D006493:Heparin; D005176:Factor XIII
keywords: Acquired hemorrhagic disorder; Anticoagulation therapy; Autoantibodies; Deep vein thrombosis; Factor XIII; Pulmonary embolism; Transglutaminase