成人T细胞急性淋巴细胞白血病的临床研究

摘要

本研究总结分析成人急性淋巴细胞白血病(T细胞型,T-ALL)的临床表现和生物学特征,比较化疗与移植组患者的疗效,以阐述影响长期生存的预后因素.2000年5月至2010年5月,我院收治的初诊成人T-ALL共计22例,根据MICM分型检查结果确诊.所有患者采用VDCLP方案诱导化疗.巩固治疗阶段,根据患者接受异基因造血干细胞移植或高强度联合化疗将患者划分为移植组或化疗组.采用SPSS 13.0统计软件分析有关数据.临床特点及疗效的比较采用卡方检验,生存分析采用生命表法及Kaplan-Meier法,采用Cox回归分析影响生存的预后因素,不同组别的差异检验采用log-rank法.结果表明:①22例患者中位年龄23.5(16 -63)岁；起病时脾肿大者15例,脾大患者无事件生存(EFS)期和总体生存(0S)期较无脾大患者明显缩短(EFS:11.6个月vs56.7个月,P=0.014;0S:14.7个月vs57.0个月,P=0.013)；中位白细胞(WBC)计数为148.82(5.51 -546.0)×109/L,其中15例起病时WBC≥80×109/L,这些患者的EFS时间和OS时间明显缩短(EFS:11.8个月vs47.1个月,P=0.021；OS:16.1个月vs 47.4个月,P=0.050)；中位血小板(Plt)计数为55.36(14 - 160)×109/L,其中6例起病时Plt≤30×109/L,这些患者EFS和OS时间较短(EFS:8.4个月vs 30.0个月,P=0.033；OS:11.4个月vs32.6个月,P=0.035).②22例中pro-T 2例,pre-T 14例,皮质T3例,髓质T3例；早期表型(pro-T&pre-T)与晚期表型(皮质和髓质T)相比,晚期表型者的EFS和OS时间明显延长(EFS:25.8个月vs 14.9个月,P=0.035；0S:28.2个月vs18.7个月,P=0.028).③染色体核型结果可供分析的19例,包括正常核型12例、异常核型7例(其中复杂核型3例,亚二倍体2例,假二倍体2例).正常核型组与异常核型组在EFS和OS时间方面没有明显差异.④诱导化疗总完全缓解(CR)率为72.7％,中位缓解时间为18.0个月.22例患者1年及3年EFS率分别为57.9％和23.0％,1年及3年OS率分别为67.1％和22.0％.化疗组16例,移植组6例,移植组比化疗组EFS和OS时间显著延长(EFS:57.8个月vs9.9个月,P=0.001;OS:57.8个月vs14.4个月,P=0.002).⑤Cox回归分析显示,在诱导化疗后采取异基因造血干细胞移植为独立的预后良好因素.结论:成人T-ALL采用强烈的诱导治疗方案可以取得较高的CR率,但单纯化疗的生存情况较差,异基因干细胞移植可以明显改善疗效.%This study was aimed to summarize and analyze the clinical features and biological characteristics of adult acute T-lymphoblastic leukemia( T-ALL), and compare the efficacy of chemotherapy and transplantation in order to explore the factors influencing the long term survival and prognosis. Twenty-two T-ALL patients, all of whom were initially diagnosed according to M1CM classification criteria from May 2000 to May 2010, were enrolled in this study. All patients received VDCLP regimen as the induction chemotherapy. In consolidation stage, some of the patients received al-logeneic bematopoietic stem cell transplantation (allo-HSCT) and the others underwent intensive chemotherapy. The clinical and laboratory parameters were summarized and the contribution to survival and efficacy was analyzed by using X2 test, Kaplan-Meier method, Cox regression analysis and log-rank test with the aid of SPSS13.0 software. The results showed that: (1) The median age of all 22 patients was 23.5 years (16-63 years). IS patients with splenomegaly had much shorter event-free survival (EFS) period (P =0.014) and overall survival (OS) period (P =0.013). Themedian white blood cell(WBC) count was 148.82(5.51 -546.0) ×l09/L. 15 cases out of them had leucocytosis (WBC &80 × 109/L) , whose EFS period (P = 0. 021) and OS time ( P = 0. 050) were reduced significantly. The similar condition was observed in 6 patients whose blood platelet(Pit) count was no more than 30 × 109/L( P = 0.033 for EFS and P =0.035 for OS, respectively); (2) Immunophenotypic analysis showed that from 22 cases 2 cases were of pro-T, 14 cases of pre-T, 3 cases of cortical-T and 3 cases of medullary-T. Supposing pro-T and pre-T as earlier period immunophenotype, cortical-T and medullary-T as advanced stage immunophenotype, there were significant differences between earlier period and advanced stage patients in terms of EFS and OS (P = 0.035 for EFS and P = 0.028 for OS, respectively); (3) Chromosome karyotype was analyzed in 19 cases at diagnosis, and among them 12 cases had normal karyofypes while abnormal karyotypes were observed in 7 cases. Correlation analysis showed that there were no significant differences between these two groups in time of EFS and OS; (4) The overall complete remission(CR) rate was 72.7% after the induction chemotherapy. The median CR period was 18.0 months. The EFS and OS rate were 57.9% and 67.1% for I-year, and 23.0% EFS rate and 22.0% OS rate for 3-years, respectively. Six patients received allo-HSCT and the average EFS time and OS time were both 57.8 months, which were significantly longer than those of the intensive chemotherapy group (P = 0. 001 and P = 0.002 for EPS and OS, respectively); (5) Cox regression analysis proved that allo-HSCT treatment was the independent favorable prognostic factor. It is concluded that higher CR rate can be achieved by using intensive induction chemotherapy in adult T ALL, but the long term survival seems poor by chemotherapy only in consolidation treatment stage. Allo-HSCT is the optimal choice to improve the prognosis and the outcome.