内容紹介
Unidentified Inflammatory Disease Induced by Azacitidine Therapy for Myelodysplastic Syndrome
Summary
We report a 73-year-old woman with myelodysplastic syndromes(MDS)of the refractory anemia with excess of blasts-1 subtype, which was diagnosed in April 2014 on the basis of cytopenia for two cell types. After completing 3 cycles of azacitidine(AZA)therapy, the patient was admitted to our hospital based on an initial presentation of high fever. During hospitalization, the high fever and increasing inflammatory reaction persisted. We reevaluated the effect of MDS in this patient and concluded that the AZA administration was successful and the MDS was extremely stable. On medical examination and inspection, the patient had an unidentified inflammatory disease. First, we treated her with high-dose steroid pulse therapy. However, the effect of the treatment was transient. Furthermore, the effects of cyclosporin A and oral steroid therapy were poor; therefore, we initiated tocilizumab administration. Nevertheless, she died of multiorgan failure. An increasing serum IL-6 level induced by the AZA therapy was later confirmed. Recent studies have reported the immunomodulatory effects stimulated by AZA therapy in MDS. This case is a valuable reminder that an unidentified inflammatory disease can be induced in the course of AZA therapy for MDS.
要旨
症例は73歳,女性。2014年4月に2系統の血球減少を認め,骨髄異形成症候群(myelodysplastic syndromes: MDS), refractory anemia with excess of blasts 1(MDS RAEB1)と診断した。9月に5-azacitidine(AZA)療法を導入し,3サイクル目を開始したところ,高熱を認めたため入院となった。入院後,高熱の持続および炎症反応の著しい上昇を認めた。MDSの再評価を施行したところAZA療法は奏効し,病勢は極めて安定していたが,原因不明の炎症性疾患を呈した。成人スティル病(adult onset Still's disease: AOSD)の診断基準を満たしたことからcyclosporin Aおよびステロイド併用療法を施行したが治療効果は乏しく,さらにtocilizumab療法を導入したが全身状態の増悪により永眠された。後日,AZA療法に伴う血中IL-6の漸増が確認された。近年,AZAが免疫系に作用する報告を認めている。本症例は,MDSに対するAZA療法の経過のなかで原因不明の炎症性疾患を呈したと推測される貴重な症例と考えた。
目次
Summary
We report a 73-year-old woman with myelodysplastic syndromes(MDS)of the refractory anemia with excess of blasts-1 subtype, which was diagnosed in April 2014 on the basis of cytopenia for two cell types. After completing 3 cycles of azacitidine(AZA)therapy, the patient was admitted to our hospital based on an initial presentation of high fever. During hospitalization, the high fever and increasing inflammatory reaction persisted. We reevaluated the effect of MDS in this patient and concluded that the AZA administration was successful and the MDS was extremely stable. On medical examination and inspection, the patient had an unidentified inflammatory disease. First, we treated her with high-dose steroid pulse therapy. However, the effect of the treatment was transient. Furthermore, the effects of cyclosporin A and oral steroid therapy were poor; therefore, we initiated tocilizumab administration. Nevertheless, she died of multiorgan failure. An increasing serum IL-6 level induced by the AZA therapy was later confirmed. Recent studies have reported the immunomodulatory effects stimulated by AZA therapy in MDS. This case is a valuable reminder that an unidentified inflammatory disease can be induced in the course of AZA therapy for MDS.
要旨
症例は73歳,女性。2014年4月に2系統の血球減少を認め,骨髄異形成症候群(myelodysplastic syndromes: MDS), refractory anemia with excess of blasts 1(MDS RAEB1)と診断した。9月に5-azacitidine(AZA)療法を導入し,3サイクル目を開始したところ,高熱を認めたため入院となった。入院後,高熱の持続および炎症反応の著しい上昇を認めた。MDSの再評価を施行したところAZA療法は奏効し,病勢は極めて安定していたが,原因不明の炎症性疾患を呈した。成人スティル病(adult onset Still's disease: AOSD)の診断基準を満たしたことからcyclosporin Aおよびステロイド併用療法を施行したが治療効果は乏しく,さらにtocilizumab療法を導入したが全身状態の増悪により永眠された。後日,AZA療法に伴う血中IL-6の漸増が確認された。近年,AZAが免疫系に作用する報告を認めている。本症例は,MDSに対するAZA療法の経過のなかで原因不明の炎症性疾患を呈したと推測される貴重な症例と考えた。