ヒグチ　トモアキ   樋口　智昭    所属   医学部 医学科（東京女子医科大学病院）    職種   寄附部門講師
論文種別	症例報告
言語種別	英語
査読の有無	査読あり
表題	A case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura.
掲載誌名	正式名：Modern rheumatology case reports 略　 称：Mod Rheumatol Case Rep ISSNコード：24725625/24725625
巻・号・頁	pp.-
著者・共著者	Motoyama Ryo, Higuchi Tomoaki, Hirahara Shinya, Konda Naoko, Yamada Risa, Watanabe Kotaro, Fujisaki Mayuko, Yamaguchi Rei, Katsumata Yasuhiro, Kawaguchi Yasushi, Harigai Masayoshi
発行年月	2023/02/08
概要	An 18-year-old Japanese woman with systemic lupus erythematosus (SLE) experienced dyspnoea, headache, tinnitus, and purpura for two weeks and was admitted to our hospital. The patient had been diagnosed with SLE and secondary immune thrombocytopenia eight years before and treated with high-dose prednisolone (PSL) and mycophenolate mofetil. Since the blood test on admission showed haemolytic anaemia with a positive direct Coombs test and anti-glycoprotein IIb/IIIa antibodies, the patient was initially diagnosed with Evans syndrome (ES). The patient was treated with pulse intravenous methylprednisolone followed by 45 mg/day prednisolone; however, the patient's platelet count did not normalise. Based on a low level of a disintegrin-like metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) activity and a high level of ADAMTS-13 inhibitors, a diagnosis of acquired thrombotic thrombocytopenic purpura (TTP) was confirmed. After undergoing therapeutic plasma exchange for six consecutive days, the patient's platelet count recovered rapidly. Although concurrent acquired TTP and ES have not been reported previously, the findings from this case highlight the importance of measuring ADAMTS-13 activity and inhibitors to rule out acquired TTP, especially when ES is refractory to glucocorticoids.
DOI	10.1093/mrcr/rxad011
PMID	36779528