タキザワ　マキコ   TAKIZAWA Makiko   滝沢　牧子    所属   埼玉医科大学  医学部 総合医療センター　医療安全管理学    職種   教授
論文種別	学術雑誌(原著)
言語種別	英語
査読の有無	査読あり
表題	Successful Management of a Patient with Autoimmune Hemorrhaphilia due to Anti-Factor XIII/13 Antibodies Complicated by Pulmonary Thromboembolism.
掲載誌名	正式名：Acta haematologica ISSNコード：0001-5792/1421-9662
掲載区分	国外
出版社	KARGER
巻・号・頁	137(3),141-147頁
著者・共著者	Yoshiyuki Ogawa,Kunio Yanagisawa,Masayoshi Souri,Masahiro Mihara,Chiaki Naito,Makiko Takizawa,Takuma Ishizaki,Takeki Mitsui,Hiroshi Handa,Tsukasa Osaki,Yoshihisa Nojima,Akitada Ichinose
発行年月	2017
概要	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.
DOI	10.1159/000455938
PMID	28380473