Horai Makiko, Satoh Shinya, Matsuo Masatoshi, Iwanaga Masako, Horio Kensuke, Jo Tatsuro, Takasaki Yumi, Kawaguchi Yasuhisa, Tsushima Hideki, Yoshida Shinichiro, Taguchi Masataka, Itonaga Hidehiro, Sawayama Yasushi, Taguchi Jun, Imaizumi Yoshitaka, Hata Tomoko, Moriuchi Yukiyoshi, Haase Detlef, Yoshiura Koh-Ichiro, Miyazaki Yasushi
Department of Haematology, Atomic Bomb Disease and Hibakusha Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
Department of Internal Medicine, Nagasaki Harbour Medical Centre City Hospital, Nagasaki, Japan.
Br J Haematol. 2018 Feb;180(3):381-390. doi: 10.1111/bjh.15050. Epub 2017 Dec 19.
The myelodysplastic syndromes (MDS) are clonal haematopoietic disorders that develop de novo and also secondary to chemotherapy and/or radiation therapy. We previously demonstrated that the risk of MDS is increased among atomic bomb survivors with significant correlation to radiation dose; however, the clinical characteristics of these survivors have not been well analysed. In this study, we investigated chromosomal abnormalities of MDS among survivors. The frequency of abnormal karyotypes was significantly higher, with more very poor risk karyotypes, according to the revised International Prognostic Scoring System, among those exposed close to the hypocentre compared with unexposed cases. However, abnormal karyotype frequency did not reflect the prognosis of exposed cases with respect to distance from the hypocentre. In addition, there was no difference in prognosis between exposed and unexposed cases. Among proximally exposed cases (<1·5 km from the hypocentre), chromosomal translocations and inversions were more frequent, and the frequency of structural alterations in chromosomes 3, 8, and 11 was significantly increased compared with unexposed cases. These results suggest that chromosomal alterations in MDS among survivors have different features compared with those in de novo or therapy-related MDS. Detailed molecular study is warranted.
骨髓增生异常综合征（MDS）是一种克隆性造血疾病，可原发发生，也可继发于化疗和/或放疗。我们之前证明，原子弹幸存者中MDS的风险增加，且与辐射剂量显著相关；然而，这些幸存者的临床特征尚未得到充分分析。在本研究中，我们调查了幸存者中MDS的染色体异常情况。根据修订后的国际预后评分系统，与未暴露者相比，在靠近爆心处暴露的人群中，异常核型的频率显著更高，且有更多预后极差的核型。然而，异常核型频率并未反映出暴露病例距爆心距离与预后的关系。此外，暴露病例和未暴露病例的预后没有差异。在近距离暴露病例（距爆心<1.5公里）中，染色体易位和倒位更为频繁，与未暴露病例相比，3号、8号和11号染色体的结构改变频率显著增加。这些结果表明，幸存者中MDS的染色体改变与原发或治疗相关MDS的染色体改变具有不同特征。有必要进行详细的分子研究。
