Harada Yasuhiko, Nagata Yasunobu, Kihara Rika, Ishikawa Yuichi, Asou Norio, Ohtake Shigeki, Miyawaki Shuichi, Sakura Toru, Ozawa Yukiyasu, Usui Noriko, Kanamori Heiwa, Ito Yoshikazu, Imai Kiyotoshi, Suehiro Youko, Kobayashi Shinichi, Kitamura Kunio, Sakaida Emiko, Onizuka Makoto, Takeshita Akihiro, Ishida Fumihiro, Suzushima Hitoshi, Ishizawa Kenichi, Naoe Tomoki, Matsumura Itaru, Miyazaki Yasushi, Ogawa Seishi, Kiyoi Hitoshi
Department of Hematology and Oncology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Department of Pathology and Tumor Biology, Graduate School of Medicine, Kyoto University, Japan; Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
Leuk Res. 2018 Mar;66:20-27. doi: 10.1016/j.leukres.2018.01.008. Epub 2018 Jan 17.
Many genetic alterations that are associated with the prognosis of acute myeloid leukemia (AML) have been identified, and several risk stratification systems based on the genetic status have been recommended. The European LeukemiaNet (ELN) first proposed the risk stratification system for AML in 2010 (ELN-2010), and recently published the revised system (ELN-2017). We validated the long-term prognosis and clinical characteristics of each ELN-2017 risk category in Japanese adult AML patients who were treated in the Japan Adult Leukemia Study Group (JALSG) AML-201 study. We demonstrated that the 3-risk category system of the ELN-2017 successfully discriminated the overall survival and complete remission rates in our cohort in comparison with the 4-risk category of the ELN-2010. However, there were still genetic categories in which stratification of patients into favorable or intermediate risk categories was controversial; the low allelic ratio of FLT3-ITD was not necessarily associated with a better prognosis in patients with FLT3-ITD, and cytogenetic abnormalities may affect the prognosis in patients with favorable genetic lesions such as NPM1 and CEBPA mutations. As many molecular targeting agents, such as FLT3 inhibitors, have been developed, we must continue to modify the genetic risk stratification system to match the progression of therapeutic strategies.
许多与急性髓系白血病（AML）预后相关的基因改变已被识别，基于基因状态的几种风险分层系统也已被推荐。欧洲白血病网（ELN）于2010年首次提出AML风险分层系统（ELN - 2010），最近又发布了修订版系统（ELN - 2017）。我们对在日本成人白血病研究组（JALSG）AML - 201研究中接受治疗的日本成年AML患者的ELN - 2017各风险类别的长期预后和临床特征进行了验证。我们证明，与ELN - 2010的4风险类别相比，ELN - 2017的3风险类别系统成功区分了我们队列中的总生存率和完全缓解率。然而，仍有一些基因类别，将患者分层为低危或中危类别存在争议；FLT3 - ITD低等位基因比率在FLT3 - ITD患者中不一定与更好的预后相关，并且细胞遗传学异常可能会影响具有有利基因病变（如NPM1和CEBPA突变）患者的预后。由于许多分子靶向药物，如FLT3抑制剂已被开发出来，我们必须继续修改基因风险分层系统以匹配治疗策略的进展。
