Wang Tong, Zhao Yan, Zhang Qing-Qing, Xu Lian-Rong
Department of Hematology, Second Hospital of Shanxi Medical University, Taiyuan 030001, Shanxi Province, China.
Department of Hematology, Second Hospital of Shanxi Medical University, Taiyuan 030001, Shanxi Province, China,E-mail:
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2020 Jun;28(3):775-780. doi: 10.19746/j.cnki.issn.1009-2137.2020.03.010.
To investigate the clinical characteristics and prognostic influencing factors of adult AML patients with MLL rearrangement.
Clinical data of 184 adult AML patients with MLL rearrangement treated in our hospital from January 2011 to December 2017 were analyzed retrospectively. The clinical features, immunophenotypic characteristics, cytogenetic characteristics, molecular biological characteristics and gene mutation characteristics were recorded, the survival and prognostic influencing factors of patients were analyzed.
Among 184 patients, 94 cases were male, 90 cases were female, median age were 36.0 years, median WBC count were 22.0×10/L, 156 cases as 84.78% for FAB typing M5, and 18 cases as 28.13% for MLL/AF9 gene positive. The median total survival time and recurrence-free survival time of 184 patients were 15.7 months and 13.3 months respectively. The cumulative total survival rate and recurrence-free survival rate by followed-up for 2 years were 36.72% and 29.33% respectively. The cumulative overall survival rate and recurrence-free survival rate of transplant recipients were significantly higher than those of non-transplant recipients by follow-up for 2 years (P＜0.05). Univariate analysis showed that age, baseline WBC count, baseline Hb levels, complete remission after one course of treatment and transplantation or no were the influencing factors of overall survival time in adult AML patients with MLL rearrangement (P＜0.05). Cox regression model multivariate analysis showed that baseline WBC count, complete remission after one course of treatment, and transplantation or no were the independent influencing factors for overall survival time in adult AML patients with MLL rearrangement（P＜0.05）.
Adult AML patients with MLL rearrangement are mostly belong to acute monocytic leukemia, and MLL/AF9 is the most common associated gene. Patients with AML and MLL rearrangement are prone to recurrence after routine chemotherapy. Allo-HSCT treatment is helpful to improve clinical prognosis of patients.
探讨成人MLL重排急性髓系白血病（AML）患者的临床特征及预后影响因素。
回顾性分析2011年1月至2017年12月在我院治疗的184例成人MLL重排AML患者的临床资料。记录其临床特征、免疫表型特征、细胞遗传学特征、分子生物学特征及基因突变特征，分析患者的生存情况及预后影响因素。
184例患者中，男性94例，女性90例，中位年龄36.0岁，中位白细胞计数22.0×10⁹/L，FAB分型M5为156例（84.78%），MLL/AF9基因阳性为18例（28.13%）。184例患者的中位总生存时间和无复发生存时间分别为15.7个月和13.3个月。随访2年的累积总生存率和无复发生存率分别为36.72%和29.33%。随访2年，移植受者的累积总生存率和无复发生存率显著高于未移植受者（P＜0.05）。单因素分析显示，年龄、基线白细胞计数、基线血红蛋白水平、一个疗程治疗后完全缓解及是否移植是成人MLL重排AML患者总生存时间的影响因素（P＜0.05）。Cox回归模型多因素分析显示，基线白细胞计数、一个疗程治疗后完全缓解及是否移植是成人MLL重排AML患者总生存时间的独立影响因素（P＜0.05）。
成人MLL重排AML患者大多属于急性单核细胞白血病，MLL/AF9是最常见的相关基因。MLL重排AML患者常规化疗后易复发。异基因造血干细胞移植治疗有助于改善患者的临床预后。
