Ishikawa Takayuki, Tohyama Kaoru, Nakao Shinji, Yoshida Yataro, Teramura Masanao, Motoji Toshiko, Takatoku Masaaki, Kurokawa Mineo, Mitani Kinuko, Uchiyama Takashi, Omine Mitsuhiro
Department of Hematology and Oncology, Graduate School ofMedicine, Kyoto University, 54 Shogoin-Kawaracho, Sakyo-ku, Kyoto 606-8507, Japan.
Int J Hematol. 2007 Aug;86(2):150-7. doi: 10.1532/IJH97.07052.
Although immunosuppressive therapy using antithymocyte globulin or cyclosporine A (CSA) is effective in selected patients with low-risk myelodysplastic syndrome, the response rates reported so far are inconsistent, and the indication of immunosuppressive therapy for myelodysplastic syndrome has not been clearly defined. We treated 20 myelodysplastic syndrome patients (17 refractory anemia cases [RA], 2 RA with excess blasts, and one RA with ringed sideroblasts) with 4 mg/kg per day of CSA for 24 weeks. Among the 19 patients evaluated, 10 showed hematologic improvement; 8 patients showed an erythroid response, 6 showed a platelet response, and one showed a neutrophil response. Most patients with hematologic improvement continued CSA thereafter, and the progressive response was observed until the latest follow-up (median, 30 months). Most toxicities associated with CSA usage were manageable, and no patient had developed acute leukemia up to this point. Short duration of illness, refractory anemia with minimal dysplasia determined by bone marrow morphology, and the presence of paroxysmal nocturnal hemoglobinuria-type cells were significantly associated with the platelet response. A minority of RA patients who did not possess such predictive variables achieved an isolated erythroid response. In conclusion, CSA may be a therapeutic option for patients with RA who do not have adverse prognostic factors.
尽管使用抗胸腺细胞球蛋白或环孢素A(CSA)进行免疫抑制治疗对部分低危骨髓增生异常综合征患者有效,但目前报道的缓解率并不一致,且骨髓增生异常综合征免疫抑制治疗的适应证尚未明确界定。我们对20例骨髓增生异常综合征患者(17例难治性贫血[RA]、2例伴有过多原始细胞的RA和1例伴有环形铁粒幼细胞的RA)给予每日4mg/kg的CSA治疗,持续24周。在评估的19例患者中,10例出现血液学改善;8例出现红系反应,6例出现血小板反应,1例出现中性粒细胞反应。大多数血液学改善的患者此后继续使用CSA,直至最新随访(中位时间30个月)均观察到进行性反应。与使用CSA相关的大多数毒性反应可控,截至目前无患者发生急性白血病。病程短、骨髓形态学显示发育异常轻微的难治性贫血以及存在阵发性夜间血红蛋白尿型细胞与血小板反应显著相关。少数不具备这些预测变量的RA患者仅出现红系反应。总之,对于没有不良预后因素的RA患者,CSA可能是一种治疗选择。