Masuda Kenta, Shiga Shuichi, Kawabata Hiroshi, Takaori-Kondo Akifumi, Ichiyama Satoshi, Kamikubo Yasuhiko
Department of Clinical Laboratory, Kyoto University Hospital, Shogoin Kawahara-cho 54, Sakyo-ku, Kyoto-shi, Kyoto, 606-8507, Japan.
Department of Hematology and Immunology, Kanazawa Medical University, Uchinadamachi Daigaku 1-1, Kahoku-gun, Ishikawa, 920-0293, Japan.
Int J Hematol. 2018 Jul;108(1):30-38. doi: 10.1007/s12185-018-2443-1. Epub 2018 Mar 29.
Myelodysplastic syndrome (MDS) is a group of clonal stem cell disorders characterized by hematopoietic insufficiency. The accurate risk stratification of patients with MDS is essential for selection of appropriate therapies. We herein conducted a retrospective cohort study to examine the prognostic value of periodic acid-Schiff (PAS) reaction-positive erythroblasts in MDS patients. We examined the PAS positivity of the bone marrow erythroblasts of 144 patients newly diagnosed with MDS; 26 (18.1%) of them had PAS-positive erythroblasts, whereas 118 (81.9%) did not. The PAS-positive group showed significantly poorer karyotypes as defined in the revised International Prognostic Scoring System (IPSS-R) and higher scores in age-adjusted IPSS-R (IPSS-RA) than the PAS-negative group. Overall survival (OS) and leukemia-free survival (LFS) were also significantly shorter in the PAS-positive group than in the PAS-negative group. Similar results were obtained when only high- and very high risk groups were analyzed using IPSS-RA. This retrospective study suggested that the PAS positivity of erythroblasts is an additional prognostic factor combined with other risk scores for OS and LFS in MDS, and our results may contribute to improved clinical decision-making and rapid risk stratification.
骨髓增生异常综合征(MDS)是一组以造血功能不全为特征的克隆性干细胞疾病。对MDS患者进行准确的风险分层对于选择合适的治疗方法至关重要。我们在此进行了一项回顾性队列研究,以检验过碘酸希夫(PAS)反应阳性的成红细胞在MDS患者中的预后价值。我们检查了144例新诊断为MDS患者的骨髓成红细胞的PAS阳性情况;其中26例(18.1%)有PAS阳性的成红细胞,而118例(81.9%)没有。与PAS阴性组相比,PAS阳性组在修订的国际预后评分系统(IPSS-R)中显示出明显更差的核型,并且在年龄调整的IPSS-R(IPSS-RA)中得分更高。PAS阳性组的总生存期(OS)和无白血病生存期(LFS)也明显短于PAS阴性组。当仅使用IPSS-RA分析高危和极高危组时,也得到了类似的结果。这项回顾性研究表明,成红细胞的PAS阳性是MDS中OS和LFS的一个与其他风险评分相结合的额外预后因素,我们的结果可能有助于改善临床决策和快速风险分层。