MLL基因重排阳性的急性白血病患者的临床特点及预后分析

发布时间：2018-01-27 16:59

本文关键词： MLL基因重排急性白血病临床特点预后　出处：《福建医科大学》2016年硕士论文　论文类型：学位论文

【摘要】：目的:探讨混合谱系白血病(Mixed Lineage Leukemia,MLL)基因重排阳性的成人及儿童急性白血病(Acute Leukemia,AL)患者的临床特点及其预后分析。方法:以2014.01.01-2015.10.31福建医科大学附属协和医院血液科收治41例MLL基因重排阳性的成人及儿童初诊AL患者为研究对象,对照组选取同期31项融合基因均阴性的77例成人及儿童初诊AL患者,其中包括全部51例成人急性髓系白血病(Acute Myeloblastic Leukemia,AML)(非M3型)患者(同时检测11项突变基因),全部14例儿童AML(非M3型)患者,及从120例儿童急性淋巴细胞白血病(Acute Lymphoblastic Leukemia ALL)中通过系统抽样随机选取的12例患者。所有AL确诊均按照MICM标准,随访时间截止至2016.02.01。回顾性分析MLL基因重排阳性AL患者的FAB分型、初诊时白细胞数、乳酸脱氢酶(LDH)、染色体等临床特点,对比组间完全缓解率(Complete Remission,CR),复发率、总体生存率(Overall Survival,OS)、无复发生存率(Relapsed-free Survival,RFS)等预后指标以期评估MLL基因重排阳性的AL患者的预后。结果:在767例初诊AL患者中有41例MLL基因重排阳性患者,阳性率为5.45%,其中成人阳性率为6.00%(28/467),儿童阳性率为4.33%(13/300)。41例MLL基因重排阳性患者中dupMLL阳性者17例,MLL/AF4者4例,MLL/AF6者2例,MLL/AF9者10例,MLL/AF10者5例,MLL/AF17者1例,MLL/ENL者2例。MLL基因重排阳性的28例成人患者均为AML,13例阳性儿童患者中,ALL与AML各占5例及8例。对比阳性组及对照组患者,二者两次化疗后总CR率分别为51.2%(21/41)、70.1%(54/77),达CR后复发率分别为33.3%(7/21)、13.0%(7/54),差别均具有显著性(P0.05)。对比成人及小儿患者,两次化疗后总CR率分别为53.6%(15/28)、46.2%(6/13),达CR后复发率分别为40.0%(6/15)、16.7%(1/6);两者总CR率及复发率的差异无统计学意义(P0.05)。对比dupMLL阳性患者及MLL基因重排其他亚型阳性患者,前者两次化疗后总CR率为47.1%(8/17),CR后复发率为25.0%(2/8);后者两次化疗后总CR率为54.2%(13/24),达CR后复发率为38.5%(5/13),两者上述差异无统计学意义(P0.05)。截止随访时间至2016.02.01,阳性组41例患者存活20例,2年OS率为(41.8±9.2)%,预计2年RFS率为(42.5±5.2)%;对照组77例患者存活54例,2年OS率为(55.6±8.7)%,预计2年RFS率为(65.3±1.4)%;二者在OS率、RFS率的差别具有显著性(P0.05)。阳性组中,28例成人患者存活12例,2年OS率为(36.2±10.5)%;13例儿童患者存活8例,预计2年OS率为(54.9±6.6)%;二者OS率差别无统计学意义(P0.05)。17例dupMLL阳性患者中存活9例,2年OS率为(45.9±14.2)%;24例MLL基因重排其他亚型阳性患者中存活11例,预计2年OS率为(23.8±3.8)%,二者OS率差别无统计学意义(P0.05)。结论:MLL基因重排阳性AL患者两次化疗的总缓解率低,且缓解后较易复发,总生存率、无复发生存率显著低于31项融合基因筛查均阴性患者。MLL基因重排阳性成人及儿童患者的缓解率及总生存率无明显差别,且dupMLL与MLL基因重排其他亚型组间比较的缓解率及总生存率亦无明显差别。
[Abstract]:Objective: to study mixed Lineage Leukemia of mixed lineage leukemia. Acute Leukemia in adults and children with positive MLL gene rearrangement. Al). Clinical features and prognosis of patients. Methods:. 41 newly diagnosed AL patients with positive MLL gene rearrangement were treated in Department of Hematology, Union Hospital, Fujian Medical University, from January 1 to October 31, 2014.The subjects were newly diagnosed AL patients. In the control group, 77 AL patients with 31 negative fusion genes were selected. All 51 adult acute myeloid leukemia patients with acute Myeloblastic Leukemia were included. Patients with AMLN (non-M3 type) (11 mutation genes were detected at the same time, all 14 cases of childhood AML3 (non-M3 type) patients. Acute Lymphoblastic Leukemia ALL from 120 children with acute lymphoblastic leukemia. All patients were diagnosed according to MICM criteria. The follow-up time was up to 2016.02.01.The FAB typing, the number of white blood cells and the lactate dehydrogenase (LDH) in AL patients with positive MLL gene rearrangement were analyzed retrospectively. The complete remission rate (CR), recurrence rate and overall survival rate of all Survival were compared between the two groups. The recurrence free survival rate was associated with Relapsed-free Survival. Results: 41 of 767 newly diagnosed AL patients were positive for MLL gene rearrangement. The positive rate was 5.45%, and the adult positive rate was 6.00% / 467%. The positive rate of MLL gene rearrangement in children was 4.33 / 300%. Of the 41 patients with positive MLL gene rearrangement, 17 were dupMLL positive, 4 were MLL / AF4, 2 were MLL / AF6. 10 cases of MLL/AF9 with MLL / AF10, 5 cases of MLL / AF17, 1 case of MLL / ENL, 2 cases of MLL / ENL gene rearrangement positive, 28 cases of adult patients were all AML. All and AML accounted for 5 cases and 8 cases respectively in 13 positive children. Compared with the control group and the positive group, the total CR rate after two chemotherapy was 51.2% and 21% / 41 respectively. The recurrence rate after CR was 33. 3 / 7 / 21 and 13.0 / 7 / 54, respectively. The difference was significant (P 0.05). Compared with the patients in adults and children, the total CR rate after two chemotherapy was 53.6 / 28 / 46.2 / 13 respectively. The recurrence rate after CR was 40.010 / 15 / 16.7 / 1 / 6 respectively. There was no significant difference in total CR rate and recurrence rate between the two groups (P 0.05). The comparison was made between the patients with dupMLL positive and the patients with MLL gene rearrangement and other subtypes. In the former, the total CR rate after two chemotherapy was 47.1% and the recurrence rate after CR was 25.0% or 2 / 8%. The total CR rate after two chemotherapy was 54.2% and the recurrence rate after CR was 38.5% or 5 / 13). There was no significant difference between the two groups (P 0.05). By the end of the follow-up period to 2016.02.01, 20 patients in the positive group survived, and the 2-year OS rate was 41.8 卤9.2%. The RFS rate was estimated to be 42.5 卤5.2% in 2 years. The survival rate of 77 patients in the control group was 54, the OS rate for 2 years was 55.6 卤8.7, and the RFS rate for 2 years was estimated to be 65.3 卤1.4; In the positive group, there were 12 cases of survival in adult patients and 36.2 卤10.5% in 2 years. The survival rate of 13 children was 8, and the OS rate of 2 years was estimated to be 54.9 卤6.6; There was no significant difference in the OS rate between the two groups. Among the 17 patients with dupMLL positive, 9 cases survived, and the 2 year OS rate was 45.9 卤14.2%. Of the 24 patients with positive MLL gene rearrangement other subtypes, 11 survived, and the 2 year OS rate was estimated to be 23.8 卤3.8%. Conclusion the overall remission rate of the two chemotherapy patients with positive rearrangement of MLL gene is low, and the overall survival rate is easy to recur after remission. There was no significant difference in remission rate and overall survival rate between adults and children with positive rearrangement of MLL gene in 31 patients with negative fusion gene screening. There was no significant difference in remission rate and overall survival rate between dupMLL and other subtypes of MLL gene rearrangement.
【学位授予单位】：福建医科大学
【学位级别】：硕士
【学位授予年份】：2016
【分类号】：R733.71

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