Kudo Daisuke, Shimizu Misayo, Kuroda Akihiro, Suyama Takuya, Shinagawa Atsushi, Ito Syusaku
Department of Hematology/Oncology, Hitachi General Hospital.
Department of Dermatology, Hitachi General Hospital.
Rinsho Ketsueki. 2017;58(6):607-612. doi: 10.11406/rinketsu.58.607.
A 66-year-old male underwent prednisolone (PSL) therapy of 13 mg/day for rheumatoid arthritis (RA). Antiphospholipid antibody syndrome, neutrophilic dermatosis (ND), and myelodysplastic syndrome (MDS) developed. Treatment of MDS required red cell concentrate transfusion, and second courses of azacitidine therapy (75 mg/m daily, intravenous injection for 7 consecutive days) led to hematologic remission. Furthermore, ND improved early after the start of azacitidine therapy, making it possible to decrease the dose of PSL. After 12th courses of azacitidine therapy, treatment was discontinued and the long-term remission of MDS and ND has been maintained. During the course, the level of matrix metalloproteinase-3, as a marker of RA, also decreased. There are few case reports of MDS in which azacitidine was effective for autoimmune diseases, including ND. We report the present case.
一名66岁男性因类风湿关节炎（RA）接受每日13毫克泼尼松龙（PSL）治疗。随后出现了抗磷脂抗体综合征、嗜中性皮病（ND）和骨髓增生异常综合征（MDS）。MDS的治疗需要输注红细胞浓缩液，阿扎胞苷治疗的第二个疗程（每日75毫克/平方米，连续7天静脉注射）使血液学缓解。此外，阿扎胞苷治疗开始后不久ND有所改善，从而有可能降低PSL的剂量。在阿扎胞苷治疗的第12个疗程后，治疗停止，MDS和ND维持长期缓解。在此过程中，作为RA标志物的基质金属蛋白酶-3水平也有所下降。关于阿扎胞苷对包括ND在内的自身免疫性疾病有效的MDS病例报告很少。我们报告了本病例。
