儿童T系急性淋巴细胞白血病的临床研究

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

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≥50×10^9／L27例（62．8％），骨髓形态学分型L232例，22例患儿出现纵隔增宽。与B系ALL患儿比较，T系ALL患儿存骨髓形态学分型L2比例、大于10岁患儿的比例和WBC≥50×10^9／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。

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 × 10^9/L. In comparison with that of B cell ALL （B-ALL） , the percentages of age older than 10 years, initial WBC count more than 50×10^9/ 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）% torT-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.