Xia J, Zhao Y, Chen F, Miao M, Qiu H Y, Ma X, Tang X W, Wang Y, Wu X J, Fu Z Z, Wu D P, Chen S N
Department of Hematology, the First Affiliated Hospital of Soochow University, National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Suzhou 215006, China the First Affiliated Hospital of Soochow University, Soochow Hongci Hematology Hospital, Suzhou 215100, China.
Department of Hematology, the First Affiliated Hospital of Soochow University, National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Suzhou 215006, China.
Zhonghua Nei Ke Za Zhi. 2023 Apr 1;62(4):410-415. doi: 10.3760/cma.j.cn112138-20220411-00264.
To investigate the clinical efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for patients with acute leukemia who are positive for the SET-NUP214 fusion gene (SET-NUP214AL). This was a retrospective case series study. Clinical data of 18 patients with SET-NUP214AL who received allo-HSCT in the First Affiliated Hospital of Soochow University and Soochow Hongci Hematology Hospital from December 2014 to October 2021 were retrospectively analyzed to investigate treatment efficacy and prognosis. The Kaplan-Meier method was used for survival analysis. Of the 18 patients, 12 were male and 6 were female, and the median age was 29 years (range, 13-55 years). There were six cases of mixed phenotype acute leukemia (three cases of myeloid/T, two cases of B/T, one case of myeloid/B/T), nine cases of acute lymphoblastic leukemia (ALL) (one case of B-ALL and eight cases of T-ALL), and three cases of acute myeloid leukemia. All patients received induction chemotherapy after diagnosis, and 17 patients achieved complete remission (CR) after chemotherapy. All patients subsequently received allo-HSCT. Pre-transplantation status: 15 patients were in the first CR, 1 patient was in the second CR, 1 was in partial remission, and 1 patient did not reach CR. All patients were successfully implanted with stem cells. The median time of granulocyte and platelet reconstitution was +12 and +13 days, respectively. With a median follow-up of 23 (4-80) months, 15 patients survived, while 3 patients died. The cause of death was recurrence of SET-NUP214AL after transplantation. After allo-HSCT, 5 patients relapsed. The estimated 3-year overall survival (OS) and relapse-free survival (RFS) rates were 83.3%±15.2% and 55.4%±20.7%, respectively. Among the 15 patients who achieved CR before transplantation, there was no significant difference in OS and RFS between haploidentical HSCT and matched sibling donor HSCT (all >0.05). Allo-HSCT can improve the prognosis and long-term survival rate of patients with SET-NUP214AL. Disease recurrence is the most important factor affecting long-term survival.
探讨异基因造血干细胞移植（allo-HSCT）治疗SET-NUP214融合基因阳性（SET-NUP214AL）急性白血病患者的临床疗效。这是一项回顾性病例系列研究。回顾性分析了2014年12月至2021年10月在苏州大学附属第一医院和苏州弘慈血液病医院接受allo-HSCT的18例SET-NUP214AL患者的临床资料，以研究治疗效果和预后。采用Kaplan-Meier法进行生存分析。18例患者中，男性12例，女性6例，中位年龄为29岁（范围13 - 55岁）。其中混合表型急性白血病6例（髓系/T 3例、B/T 2例、髓系/B/T 1例），急性淋巴细胞白血病（ALL）9例（B-ALL 1例、T-ALL 8例），急性髓系白血病3例。所有患者确诊后均接受诱导化疗，17例患者化疗后达到完全缓解（CR）。所有患者随后均接受allo-HSCT。移植前状态：15例处于首次CR，1例处于第二次CR，1例部分缓解，1例未达到CR。所有患者均成功植入干细胞。粒细胞和血小板重建的中位时间分别为+12天和+13天。中位随访23（4 - 80）个月，15例患者存活，3例患者死亡。死亡原因是移植后SET-NUP214AL复发。allo-HSCT后，5例患者复发。估计3年总生存（OS）率和无复发生存（RFS）率分别为83.3%±15.2%和55.4%±20.7%。在移植前达到CR的15例患者中，单倍体HSCT与同胞全相合供者HSCT的OS和RFS无显著差异（均>0.05）。allo-HSCT可改善SET-NUP214AL患者的预后和长期生存率。疾病复发是影响长期生存的最重要因素。
