儿童高危型急性淋巴细胞白血病的危险因素及疗效

目的 探讨儿童高危ALL的危险因素及疗效,改善ALL患儿的预后.方法 回顾性分析本院2004年10月-2007年12月初诊ALL患儿的临床资料,按2004年全国小儿血液病会议通过的儿童ALL诊疗建议,具有危险因素的41例患儿进入研究.发病年龄8~12个月或10~14岁者共21例,白细胞≥50×109 L-1者20例(48.78%),初发时伴髓外浸润15例(36.59%),T淋巴细胞性白血病10例(24.39%),有不利的细胞遗传学改变5例(12.20%),窗口治疗不敏感12例(29.27%),2个疗程未获缓解1例(2.44%).以2006年1月为界,此前应用新华-99方案化疗,此后用上海儿童白血病协作组2005方案(ALL-2005方案)进行化疗.应用SPSS 13.0软件进行统计学分析.结果 随访至2010年6月,中位随访时间32.08个月(2~68个月).40例经1个疗程诱导后缓解,缓解率97.56%;10例(24.39%)复发,3例(7.32%)在治疗过程中死于感染.预期30个月无事件生存率(EFS)和总生存率(OS)分别为69.60%和73.30%.窗口治疗不敏感和诱导6周未获缓解2个危险因素作为单变量对EFS和OS影响有明显差异.结论 评判ALL的危险因素对选择治疗方案和预后判断均非常重要.强烈的联合化疗、造血干细胞移植作为补充手段治疗儿童高危白血病,能够获得较好的疗效.

Risk Factors and Curative Effect in Children with High-Risk Acute Lymphoblastic Leukemia

Objective To evaluate the risk factors and efficacy of high-risk(HR) acute lymphoblastic leukemia(ALL) in children in order to improve the prognosis. Methods Forty-one patients in Shanghai Children′s Hospital Affiliated to Shanghai Jiaotong University from Oct.2004 to Dec.2007 were newly diagnosed with ALL,diagnostic criteria of HR ALL was based on the protocol suggested by the Pediatric Hematology Group of China Medical Association in 2004.There were 21 cases(51.22%) aged between 0-1 year or 10-14 years,20 cases(48.78%) with high white blood cell count(≥50×109 L-1) at diagnosis,15 cases(36.59%) with extramedullary infiltration,10 cases(24.39%) of T-lineage,5 cases(12.20%) with adverse chromosomal abnormalities,12 cases(29.27%) with prednisone-poor respon-ders(PPR),and 1 case(2.44%) with non-remission(NR) after 2 courses of treatment.The patients diagnosed before Jan.2006 were treated with protocols Xinhua-99(XH-99),while the others used 2005 protocols of Shanghai Children Leukemia Group(protocols ALL-2005).All data were analyzed using SPSS 13.0 software. Results Followed up to Jun.2010,the median follow-up time was 32.08 months(from 2 to 68 months).Forty cases had remitted after 1 course of induction treatment,and the remission rate was 97.56%. During the treatment,10 patients(24.39%)relapsed,and 3 cases(7.32%)died of infection.The 30 months probability of event-free survival(EFS) and the overall survival(OS)were 69.60% and 73.30%,respectively.It indicated that NR in 6 weeks or PPR was an adverse independent risk factor for EFS and OS. Conclusions Risk factor classification is important for individual therapy,treatment decision and prognosis.Intense chemotherapy added with hematopoietic stem cell transplantation should timely yield better outcomes for high risk patients.