2岁以下婴幼儿急性淋巴细胞白血病41例  

Analysis of 41 cases at the age less than 2 year old with acute lymphoblasfic leukemia

作　　者：马晓莉[1] 吴敏媛[1] 张永红[1] 石慧文[1] 谢静[1] 王彬[1] 郭海英[1] 金眉[1] 张瑞东[1] 胡亚美[1] 

机构地区：[1]首都医科大学附属北京儿童医院血液中心,北京100045

出　　处：《白血病．淋巴瘤》2008年第6期415-417,共3页Journal of Leukemia & Lymphoma

摘　　要：目的总结2岁以下婴幼儿急性淋巴细胞白血病（ALL）的生物学特征。方法回顾性分析1996年1月至2005年12月在北京儿童医院诊治的2岁以下ALL患儿的临床资料及治疗效果，随访至2006年8月31日。结果共41例患儿，占同期ALL的3．9％。诊断后治疗者28例。诊断时年龄≤12个月11例，白细胞数大于20×10^9／L21例，中枢神经系统侵犯者5例，骨髓免疫学均为B细胞来源，其中带有髓系标志22例，17例存在染色体易位，其中7例为11q23易位形成的融合基因。诊断后治疗的患儿，于诱导治疗第28天骨髓完全缓解率100％，复发4例，复发时间为缓解后9—34个月（中位14个月），4例复发患儿均为高白细胞，且3例具有11q23形成的融合基因。22／28例患儿生存并随访至今，随访时间8～124个月（中位39个月）；持续完全缓解时间1～124个月（中位36．5个月）。结论2岁以下婴幼儿ALL的生物学特点明显，临床多表现为高白细胞，骨髓免疫学以B细胞型为主，且髓系相关抗原联合表达的频率很高，染色体以11q23易位形成的融合基因为多见，骨髓复发仍然是治疗失败的重要原因，伴有11q23易位形成的融合基因者预后差。Objective Reports of the clinical feature, immunophenotype and cytogenetic trait for children at age of less than 2 year old with newly diagnosed acute lymphoblastic leukemia （ALL）. Methods Between 1996.1-2005.12 in Beijing Children＇ s Hospital, children less than 2 year old with ALL were enrolled. Using retrospective methods, we collected and studied the clinical data, following up till to August 31, 2006. Results There were 41 children with ALL at age of less than 2 year old, accounting for 3.9% of all in the same time, including 11 for the age less than 12 months old, 21 with WBC more than 20×10^9/L, 5 with center nervous system（CNS） leukemia. All cases are B-cell immunophenotype, 22 with immunophenotypic coexpression of myeloid associated antigens, 17 cytogenetic abnormalities and fusion gene positive, among them, 7 involving ALL-1/MLL 11q23 gene rearrangements. Among 41 cases, 28 cases were given combined intensive chemotherapy and achieved complete remission （CR） with induction therapy for 28 days, 4 cases were relapsed from CR 9-34 months （medium 18 months）. 22 cases were follow-up continuously during 8 to 124 months （medium 39 months）, continue complete remission （CCR） from 1 to 124 months （medium36.5 months）. Conclusion Children with ALL at the age less than 2 year old display unique biological features as compared with older children, is associated with a high leukocyte count at presentation and CNS involvement. The immunophenotype is usually B-lineage and is characterized by the co-expression of myeloid-associated antigens, and presence of involving ALL-1/MLL 11q23 gene rearrangements. Marrow relapse remains the primary mode of failure. Cytogenetic abnormalities involving ALL-1/MLL 11q23 gene rearrangements with ALL have a very poor outcome.

关 键 词：儿童 白血病 淋巴细胞 急性 生物学特点 

分 类 号：R733.71[医药卫生—肿瘤]