Wang Z, Xu M Z, Chen Y F, Xue F, Zhang L, Hu Y M, Li C W, Li S Z, Wang J X, Mi Y C
State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Instituteof Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2019 Dec 14;40(12):1008-1014. doi: 10.3760/cma.j.issn.0253-2727.2019.12.007.
To investigate the clinical characteristics, diagnosis, treatment and prognosis of therapy-related myeloid neoplasms (t-MNs) after successful treatment for acute promyelocytic leukemia (APL) . Clinical data of 4 patients, diagnosed as t-MNs secondary to APL at Hematology Hospital of Chinese Academy of Medical Sciences from October 2012 to January 2019, were collected retrospectively. T-MNs related literature was reviewed. The 4 cases were all females, with the median age 42 (range 40-53) years old at the diagnosis of APL. Regarding the induction and consolidation regimens, 3 patients received all-trans retinoid acid (ATRA) and arsenic trioxide (ATO) combined with anthracycline/anthraquinone and/or cytosine. One patient only received ATRA and other auxiliary drugs. Alkylating agents were not administrated. The 4 patients developed t-MNs 40 to 43 months after complete remission (CR) of APL, including 1 case of therapy-related myelodysplastic syndrome (t-MDS) and 3 cases of acute myeloid leukemia (t-AML) . The PML-RARα fusion genes were all negative when t-MNs developed. The three patients with t-AML were treated with 3 to 4 re-induction regimens, one of whom underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) after complete remission (CR) . One patient with t-MDS received hypomethylating agents. After a median follow-up of 54.5 (48-62) months, 2 patients with t-AML died, the median overall survival after t-MN was 12 (5-18) months. From 1989 to 2018, a total of 63 t-MN cases were reported in the literature. Therefore, 67 cases were analyzed when four patients in our center were added, including 27 males and 40 females with median age 52.5 (15-76) years. The median latency was 39 (12-126) months and the median overall survival after diagnosis of t-MN was 10 (1-39) months. Although rare, t-MNs may occur after successful control of APL. There are no existing guidelines for prevention and treatment of t-MNs, which have very poor prognosis. If cytopenia or other abnormalities of peripheral blood cells develop after 3 years of APL, t-MNs should be considered as a differential diagnosis.
探讨急性早幼粒细胞白血病（APL）成功治疗后治疗相关髓系肿瘤（t-MNs）的临床特征、诊断、治疗及预后。回顾性收集2012年10月至2019年1月在中国医学科学院血液病医院诊断为继发于APL的t-MNs的4例患者的临床资料。复习t-MNs相关文献。4例均为女性，诊断APL时中位年龄42岁（范围40 - 53岁）。诱导和巩固方案方面，3例患者接受全反式维甲酸（ATRA）、三氧化二砷（ATO）联合蒽环类/蒽醌类和/或阿糖胞苷治疗。1例患者仅接受ATRA及其他辅助药物治疗，未使用烷化剂。4例患者在APL完全缓解（CR）后40至43个月发生t-MNs，其中1例为治疗相关骨髓增生异常综合征（t-MDS），3例为急性髓系白血病（t-AML）。发生t-MNs时PML-RARα融合基因均为阴性。3例t-AML患者接受3至4次再诱导方案治疗，其中1例在完全缓解（CR）后接受了异基因造血干细胞移植（allo-HSCT）。1例t-MDS患者接受了去甲基化药物治疗。中位随访54.5（48 - 62）个月后，2例t-AML患者死亡，t-MN后的中位总生存期为12（5 - 18）个月。1989年至,2018年，文献共报道63例t-MN病例。加上本中心4例患者后共分析67例，其中男性27例，女性40例，中位年龄52.5（15 - 76）岁。中位潜伏期为39（12 - 126）个月，t-MN诊断后的中位总生存期为10（1 - 39）个月。虽然罕见，但t-MNs可能在APL成功控制后发生。目前尚无t-MNs的防治指南，其预后很差。如果APL 3年后出现血细胞减少或外周血细胞其他异常，应考虑t-MNs进行鉴别诊断。
