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  本文關(guān)鍵詞： MLL基因重排 急性白血病 臨床特點 預(yù)后　出處：《福建醫(yī)科大學(xué)》2016年碩士論文　論文類型：學(xué)位論文

【摘要】：目的:探討混合譜系白血病(Mixed Lineage Leukemia,MLL)基因重排陽性的成人及兒童急性白血病(Acute Leukemia,AL)患者的臨床特點及其預(yù)后分析。方法:以2014.01.01-2015.10.31福建醫(yī)科大學(xué)附屬協(xié)和醫(yī)院血液科收治41例MLL基因重排陽性的成人及兒童初診AL患者為研究對象,對照組選取同期31項融合基因均陰性的77例成人及兒童初診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(同時檢測11項突變基因),全部14例兒童AML(非M3型)患者,及從120例兒童急性淋巴細(xì)胞白血病(Acute Lymphoblastic Leukemia ALL)中通過系統(tǒng)抽樣隨機(jī)選取的12例患者。所有AL確診均按照MICM標(biāo)準(zhǔn),隨訪時間截止至2016.02.01�；仡櫺苑治鯩LL基因重排陽性AL患者的FAB分型、初診時白細(xì)胞數(shù)、乳酸脫氫酶(LDH)、染色體等臨床特點,對比組間完全緩解率(Complete Remission,CR),復(fù)發(fā)率、總體生存率(Overall Survival,OS)、無復(fù)發(fā)生存率(Relapsed-free Survival,RFS)等預(yù)后指標(biāo)以期評估MLL基因重排陽性的AL患者的預(yù)后。結(jié)果:在767例初診AL患者中有41例MLL基因重排陽性患者,陽性率為5.45%,其中成人陽性率為6.00%(28/467),兒童陽性率為4.33%(13/300)。41例MLL基因重排陽性患者中dupMLL陽性者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基因重排陽性的28例成人患者均為AML,13例陽性兒童患者中,ALL與AML各占5例及8例。對比陽性組及對照組患者,二者兩次化療后總CR率分別為51.2%(21/41)、70.1%(54/77),達(dá)CR后復(fù)發(fā)率分別為33.3%(7/21)、13.0%(7/54),差別均具有顯著性(P0.05)。對比成人及小兒患者,兩次化療后總CR率分別為53.6%(15/28)、46.2%(6/13),達(dá)CR后復(fù)發(fā)率分別為40.0%(6/15)、16.7%(1/6);兩者總CR率及復(fù)發(fā)率的差異無統(tǒng)計學(xué)意義(P0.05)。對比dupMLL陽性患者及MLL基因重排其他亞型陽性患者,前者兩次化療后總CR率為47.1%(8/17),CR后復(fù)發(fā)率為25.0%(2/8);后者兩次化療后總CR率為54.2%(13/24),達(dá)CR后復(fù)發(fā)率為38.5%(5/13),兩者上述差異無統(tǒng)計學(xué)意義(P0.05)。截止隨訪時間至2016.02.01,陽性組41例患者存活20例,2年OS率為(41.8±9.2)%,預(yù)計2年RFS率為(42.5±5.2)%;對照組77例患者存活54例,2年OS率為(55.6±8.7)%,預(yù)計2年RFS率為(65.3±1.4)%;二者在OS率、RFS率的差別具有顯著性(P0.05)。陽性組中,28例成人患者存活12例,2年OS率為(36.2±10.5)%;13例兒童患者存活8例,預(yù)計2年OS率為(54.9±6.6)%;二者OS率差別無統(tǒng)計學(xué)意義(P0.05)。17例dupMLL陽性患者中存活9例,2年OS率為(45.9±14.2)%;24例MLL基因重排其他亞型陽性患者中存活11例,預(yù)計2年OS率為(23.8±3.8)%,二者OS率差別無統(tǒng)計學(xué)意義(P0.05)。結(jié)論:MLL基因重排陽性AL患者兩次化療的總緩解率低,且緩解后較易復(fù)發(fā),總生存率、無復(fù)發(fā)生存率顯著低于31項融合基因篩查均陰性患者。MLL基因重排陽性成人及兒童患者的緩解率及總生存率無明顯差別,且dupMLL與MLL基因重排其他亞型組間比較的緩解率及總生存率亦無明顯差別。
[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 鹵9.2%. The RFS rate was estimated to be 42.5 鹵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 鹵8.7, and the RFS rate for 2 years was estimated to be 65.3 鹵1.4; In the positive group, there were 12 cases of survival in adult patients and 36.2 鹵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 鹵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 鹵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 鹵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.
【學(xué)位授予單位】：福建醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2016
【分類號】：R733.71

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