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Title: [Characteristics and prognosis in adult acute myeloid leukemia patients with MLL gene rearrangements].
Author: Gong XY, Wang Y, Liu BC, Wei H, Li CW, Li QH, Zhao JW, Zhou CL, Lin D, Liu KQ, Wei SN, Gong BF, Zhang GJ, Liu YT, Zhao XL, Li Y, Gu RX, Qiu SW, Mi YC, Wang JX.
Journal: Zhonghua Xue Ye Xue Za Zhi; 2018 Jan 14; 39(1):9-14. PubMed ID: 29551026.
Abstract:
Objective: To analyze the clinical and laboratory characteristics, and prognosis of adult acute myeloid leukemia (AML) patients with MLL gene rearrangements. Methods: The medical records of 92 adult AML patients with MLL gene rearrangements from January 2010 to December 2016 were retrospectively analyzed. Results: 92 cases (6.5%) with MLL gene rearrangements were identified in 1 417 adult AML (Non-M(3)) patients, the median age of the patients was 35.5 years (15 to 64 years old) with an equal sex ratio, the median WBC were 21.00(0.42-404.76)×10(9)/L, and 78 patients (84.8%) were acute monoblastic leukemia according to FAB classification. Eleven common partner genes were detected in 32 patients, 9 cases (28.1%) were MLL/AF9(+), 5 cases (15.6%) were MLL/AF6(+), 5 cases (15.6%) were MLL/ELL(+), 2 cases (6.3%) were MLL/AF10(+), 1 case (3.1%) was MLL/SETP6(+), and the remaining 10 patients' partner genes weren't identified. Of 92 patients, 83 cases with a median follow-up of 10.3 (0.3-74.0) months were included for the prognosis analysis, the complete remission (CR) rate was 85.5% (71/83), the median overall survival (OS) and relapse free survival (RFS) were 15.4 and 13.1 months, respectively. Two-year OS and RFS were 36.6% and 29.5%, respectively. Of 31 patients underwent allogeneic hematopoietic stem-cell transplantation (allo-HSCT), two-year OS and RFS for patients received and non-received allo-HSCT were 57.9% and 21.4%, 52.7% and 14.9%, respectively (P<0.001). Among patients with partner genes tested, 9 of 32 cases (28.1%) were MLL/AF9(+), the median follow-up was 6.0(4.1-20.7) months. 3 patients with MLL/AF9 underwent allo-HSCT. 23 cases (71.9%) were non- MLL/AF9(+), the median follow-up was 7.8 (0.3-26.6) months. 14 patients (60.1%) with non-MLL/AF9 underwent allo-HSCT. One-year OS for patients with MLL/AF9 and non-MLL/AF9 were 38.1% and 55.5%, respectively (P=0.688). Multivariate analysis revealed that high WBC (RR=1.825, 95% CI 1.022-3.259, P=0.042), one cycle to achieve CR (RR=0.130, 95% CI 0.063-0.267, P<0.001), post-remission treatment with allo-HSCT (RR=0.169, 95% CI 0.079-0.362, P<0.001) were independent prognostic factors affecting OS. Conclusions: AML with MLL gene rearrangements was closely associated with monocytic differentiation, and MLL/AF9 was the most frequent partner gene. Conventional chemotherapy produced a high response rate, but likely to relapse, allo-HSCT may have the potential to further improve the prognosis of this group of patients. 目的： 分析MLL基因重排成人急性髓系白血病（AML）的临床、实验室特征及预后情况。 方法： 回顾性分析2010年1月至2016年12月确诊的92例MLL基因重排成人AML患者的临床和实验室资料。 结果： 1 417例成人AML（不包括急性早幼粒细胞白血病，均采用FISH方法进行了MLL基因重排分析）患者中检出92例（6.5%）MLL基因重排患者，男女性别比为1∶1，诊断时中位年龄为35.5（15~64）岁，中位WBC 21.00（0.42~404.76）×10(9)/L。按FAB分型标准，78例（84.8%）患者属于急性单核细胞白血病。32例患者检测了11种MLL常见伙伴基因，其中MLL/AF9阳性9例（28.1%），MLL/AF6阳性5例（15.6%），MLL/ELL阳性5例（15.6%），MLL/AF10阳性2例（6.3%），MLL/SETP6阳性1例（3.1%），余10例（31.3%）患者的伙伴基因未知。83例患者可进行疗效分析，中位随访时间为10.3（0.3~74.0）个月，完全缓解（CR）率为85.5%，中位总生存（OS）和无复发生存（RFS）时间分别为15.4和13.1个月，2年OS和RFS率分别为36.6%和29.5%。31例患者进行了异基因造血干细胞移植（allo-HSCT），移植患者的2年OS和RFS率分别为57.9%和52.7%，未移植患者分别为21.4%和14.9%，差异均有统计学意义（P值均<0.001）。进行伙伴基因检测的患者中，9例MLL/AF9阳性患者中位随访时间为6.0（4.1~20.7）个月，3例（33.3%）进行了allo-HSCT；23例非MLL/AF9阳性患者中位随访时间为7.8（0.3~26.6）个月，14例（60.1%）进行了allo-HSCT。两组患者的1年OS率分别为38.1%和55.5%，差异无统计学意义（P=0.688）。多因素分析显示起病时WBC（RR=1.825，95% CI 1.022~3.259，P=0.042）、是否1个疗程达CR（RR=0.130，95% CI 0.063~0.267，P<0.001）以及是否移植（RR=0.169，95% CI 0.079~0.362，P<0.001）为影响MLL基因重排AML患者OS的独立预后因素。 结论： MLL基因重排成人AML多见于急性单核细胞白血病，MLL/AF9是最常见的伙伴基因。该类型白血病常规化疗虽然缓解率尚可，但极易复发，allo-HSCT可以改善其预后。.

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