Characteristics and prognosis in adult acute myeloid leukemia patients with MLL gene rearrangements
10.3760/cma.j.issn.0253-2727.2018.01.003
- VernacularTitle: MLL基因重排成人急性髓系白血病的特征和预后分析
- Author:
Xiaoyuan GONG
1
;
Ying WANG
;
Bingcheng LIU
;
Hui WEI
;
Chengwen LI
;
Qinghua LI
;
Jiawei ZHAO
;
Chunlin ZHOU
;
Dong LIN
;
Kaiqi LIU
;
Shuning WEI
;
Benfa GONG
;
Guangji ZHANG
;
Yuntao LIU
;
Xingli ZHAO
;
Yan LI
;
Runxia GU
;
Shaowei QIU
;
Yingchang MI
;
Jianxiang WANG
Author Information
1. State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
- Publication Type:Journal Article
- Keywords:
Leukemia, myeloid, acute;
MLL rearrangement;
Prognosis
- From:
Chinese Journal of Hematology
2018;39(1):9-14
- CountryChina
- Language:Chinese
-
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-M3) 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)×109/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.
