Kawabata Hiroshi, Tohyama Kaoru, Matsuda Akira, Araseki Kayano, Hata Tomoko, Suzuki Takahiro, Kayano Hidekazu, Shimbo Kei, Zaike Yuji, Usuki Kensuke, Chiba Shigeru, Ishikawa Takayuki, Arima Nobuyoshi, Nogawa Masaharu, Ohta Akiko, Miyazaki Yasushi, Mitani Kinuko, Ozawa Keiya, Arai Shunya, Kurokawa Mineo, Takaori-Kondo Akifumi
Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Department of Hematology and Immunology, Kanazawa Medical University, Uchinada, Ishikawa, 920-0293, Japan.
Int J Hematol. 2017 Sep;106(3):375-384. doi: 10.1007/s12185-017-2250-0. Epub 2017 May 11.
The Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes has been conducting prospective registration, central review, and follow-up study for patients with aplastic anemia and myelodysplastic syndrome (MDS) since 2006. Using this database, we retrospectively analyzed the prognosis of patients with MDS. As of May 2016, 351 cases were registered in this database, 186 of which were eligible for the present study. Kaplan-Meier analysis showed that overall survival (OS) curves of the five risk categories stipulated by the revised international prognostic scoring system (IPSS-R) were reasonably separated. 2-year OS rates for the very low-, low-, intermediate-, high-, and very high-risk categories were 95, 89, 79, 35, and 12%, respectively. In the same categories, incidence of leukemic transformation at 2 years was 0, 10, 8, 56, and 40%, respectively. Multivariate analysis revealed that male sex, low platelet counts, increased blast percentage (>2%), and high-risk karyotype abnormalities were independent risk factors for poor OS. Based on these data, we classified Japanese MDS patients who were classified as intermediate-risk in IPSS-R, into the lower risk MDS category, highlighting the need for careful assessment of treatments within low- and high-risk treatment protocols.
自2006年起，日本特发性骨髓衰竭综合征国家研究小组一直在对再生障碍性贫血和骨髓增生异常综合征（MDS）患者进行前瞻性登记、集中审查和随访研究。利用该数据库，我们回顾性分析了MDS患者的预后情况。截至2016年5月，该数据库共登记了351例病例，其中186例符合本研究要求。Kaplan-Meier分析显示，修订后的国际预后评分系统（IPSS-R）规定的五个风险类别的总生存（OS）曲线有合理区分。极低、低、中、高和极高风险类别的2年OS率分别为95%、89%、79%、35%和12%。在相同类别中，2年时白血病转化的发生率分别为0、10%、8%、56%和40%。多变量分析显示，男性、血小板计数低、原始细胞百分比增加（>2%）和高危核型异常是OS不良的独立危险因素。基于这些数据，我们将IPSS-R分类为中危的日本MDS患者重新分类为低危MDS类别，强调在低危和高危治疗方案中需要仔细评估治疗方法。
