Matsuo Masatoshi, Iwanaga Masako, Kondo Hisayoshi, Soda Midori, Jo Tatsuro, Horio Kensuke, Takasaki Yumi, Kawaguchi Yasuhisa, Tsushima Hideki, Imaizumi Yoshitaka, Imanishi Daisuke, Taguchi Jun, Sawayama Yasushi, Hata Tomoko, Miyazaki Yasushi
Department of Hematology, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan.
Department of Hematology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Cancer Sci. 2016 Oct;107(10):1484-1491. doi: 10.1111/cas.13025. Epub 2016 Sep 2.
There is evidence that radiation exposure is a causative factor of myelodysplastic syndromes (MDS). However, little is known about whether radiation exposure is also a prognostic factor of MDS. We investigated the impact of radiation exposure on the prognosis of MDS in Nagasaki atomic bomb survivors using the International Prognostic Scoring System (IPSS) and the revised version (IPSS-R). Subjects were 140 patients with primary MDS diagnosed between 1985 and 2011 and evaluable for IPSS, IPSS-R, and exposure distance. Of those, 31 were exposed at <1.5 km, 35 at 1.5-2.99 km, and 74 at ≥3.0 km. By the end of March 2014, 47 patients (34%) progressed to overt leukemia and 106 (75.7%) died. By comparing with patients exposed at ≥3.0 km, those exposed at <1.5 km had significantly higher frequencies of abnormal chromosome (P = 0.02), intermediate/poor IPSS, and intermediate/poor/very poor IPSS-R cytogenetic category (P = 0.0001, and P < 0.0001, respectively). As with de novo MDS, multivariate Cox regression analyses revealed that cytogenetic abnormalities, IPSS karyotype, and IPSS-R cytogenetics were significantly associated with poor survival, and cumulative incidence of leukemic transformation in MDS among atomic bomb survivors, but exposure distance was not associated with any poor outcomes. These suggest that exposure to the greater dose of atomic bomb radiation is associated with developing poor cytogenetic abnormalities in MDS, which might consequently lead to overt leukemia among atomic bomb survivors.
有证据表明辐射暴露是骨髓增生异常综合征（MDS）的一个致病因素。然而，关于辐射暴露是否也是MDS的一个预后因素，人们知之甚少。我们使用国际预后评分系统（IPSS）及其修订版（IPSS-R），调查了辐射暴露对长崎原子弹爆炸幸存者中MDS预后的影响。研究对象为140例在1985年至2011年间确诊的原发性MDS患者，这些患者可进行IPSS、IPSS-R及暴露距离评估。其中，31例在<1.5公里处暴露，35例在1.5 - 2.99公里处暴露，74例在≥3.0公里处暴露。截至2014年3月底，47例患者（34%）进展为明显白血病，106例（75.7%）死亡。与在≥3.0公里处暴露的患者相比，在<1.5公里处暴露的患者染色体异常频率显著更高（P = 0.02），IPSS为中等/不良，IPSS-R细胞遗传学类别为中等/不良/极差（分别为P = 0.0001和P < 0.0001）。与原发性MDS一样，多因素Cox回归分析显示，细胞遗传学异常、IPSS核型和IPSS-R细胞遗传学与原子弹爆炸幸存者中MDS的不良生存及白血病转化累积发生率显著相关，但暴露距离与任何不良结局均无关联。这些结果表明，暴露于更高剂量的原子弹辐射与MDS中出现不良细胞遗传学异常有关，这可能进而导致原子弹爆炸幸存者发生明显白血病。
