We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Successful Management of a Patient with Autoimmune Hemorrhaphilia due to Anti-Factor XIII/13 Antibodies Complicated by Pulmonary Thromboembolism.
- Authors
Ogawa, Yoshiyuki; Yanagisawa, Kunio; Souri, Masayoshi; Mihara, Masahiro; Naito, Chiaki; Takizawa, Makiko; Ishizaki, Takuma; Mitsui, Takeki; Handa, Hiroshi; Osaki, Tsukasa; Nojima, Yoshihisa; Ichinose, akitada
- Abstract
Autoimmune hemophilia-like disease (hemorrhaphilia) due to anti-factor XIII (FXIII) antibodies (AH13) is a very rare, lifethreatening 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.
- Subjects
HEMORRHAGIC diseases; AUTOANTIBODIES; VENOUS thrombosis; BLOOD coagulation factor XIII; PULMONARY embolism; TRANSGLUTAMINASES
- Publication
Acta Haematologica, 2017, Vol 137, Issue 3, p141
- ISSN
0001-5792
- Publication type
Article
- DOI
10.1159/000455938