儿童T系急性淋巴细胞白血病的临床研究

目的 研究T系急性淋巴细胞白血病(ALL)患儿的临床与预后特征.方法 对1999年1月至2005年4月采用ALL-XH-99方案治疗的305例ALL患儿进行细胞形态学、免疫学、细胞遗传学和分子遗传学分型,并按型分层治疗.结果 在305例ALL患儿中T系ALL患儿43例,其中男34例(79.1%),平均年龄7.8(2.2～16.4)岁,大于10岁的患儿29例(67.4%),中危和高危组患儿分别为11例和32例,WBC≥50×109/L 27例(62.8%),骨髓形态学分型L2 32例,22例患儿出现纵隔增宽.与B系ALL患儿比较,T系ALL患儿在骨髓形态学分型L2比例、大于10岁患儿的比例和WBC≥50×109/L的比例差异均有统计学意义(P<0.05).在诱导缓解治疗中,T系ALL患儿泼尼松窗口试验反应好和第19天骨髓未达缓解的比例分别为62.9%和57.9%,与B系ALL患儿比较,差异均有统计学意义(P值均<0.01).有14例T系ALL患儿复发,完全缓解至复发时间为(1.2±1.5)年.T系ALL患儿的8年无事件生存(EFS)率、无复发生存(RFS)率和总生存(OS)率分别为(40.2±10.1)%、(51.4±11.6)%和(49.8±9.9)%,而B系ALL患儿的8年EFS率、RFS率和OS率分别为(72.1±3.0)%、(83.2±2.7)%和(76.6±2.9)%(P值均<0.01).结论 T系ALL患儿在年龄、白细胞计数和骨髓形态学分型上和B系ALL患儿存在差异,早期治疗反应以及远期疗效较B系ALL患儿差.

ALL-XH-99 protocol in the treatment of childhood T-cell acute lymphoblastic leukemia

Objective To analyze the incidence, clinical characteristics and prognosis of childhood T-cell acute lymphoblastic leukemia (T-ALL). Methods From January 1999 to April 2005, 305 patients with newly diagnosed ALL were enrolled in protocol ALL-XH-99. The clinical characteristics of these children were analysed. Results Of 305 childhood ALL patients, 43 were T-ALL There were 34 males among the 43 T-ALLs. The mean age at diagnosis was 7.8 (2.2 to 16.4) years, 29 (67.4%) cases of them were older than 10 years, and 27(62.8%) cases had initial WBC count more than 50 x 109/L. In comparison with that of B cell ALL (B-ALL), the percentages of age older than 10 years, initial WBC count more than 50×109/L, prednisone poor response (PPR), and failed to achieve remission at day 19 of induction chemotherapy in the T-ALLs were all higher. No statistic difference was found in sex between them. The eight-year event-free survival (EFS), relapse-free survival (RFS) and overall survival (OS) were (40.2±10.1)%, (51.4±11.6)% and (49.8±9.9)% for T-ALL, and (72.1±3.0)%, (83.2±2.7)%, and (76.6±2.9)% for B-ALL, respectively, being differed significantly between the two types of ALL(P < 0.01). Conclusion There were statistic differences between T-cell and B-cell childhood ALLs in age, initial WBC count, early re-sponse to therapy, and eight-year EFS and RFS. Childhood T-ALL was associated with a worse prognosis than other sub-types of childhood ALL.