497例儿童少年急性白血病髓外浸润特点

Characteristerics of extramedullary infiltration in 497 children and adolescents with acute leukemia

目的 分析儿童少年急性白血病 (AL)髓外浸润临床特点 ,指导临床治疗 ,提高长期生存率。方法 回顾性分析 4 97例儿童少年AL ,为 1 996年 5月～ 2 0 0 3年 5月我院初诊住院的急性白血病患儿。结果 1 7岁以下儿童少年AL中 ,ALL占 6 3% ,ANLL 33%。ALL初诊者髓外浸润中以纵隔浸润最多见 ,并多见于T -ALL患儿。髓外浸润在ALL高危组中的发生率最高。ANLL初诊时的髓外浸润常见部位为CNS、皮肤、眼部、胸膜等 ,多发生于M2b、M4 和M5型患儿。初诊时CNS浸润ANLL比ALL常见 ,中位年龄 1 2岁 ,男性高白细胞者多发 ,发生CNS浸润最常见的是M2b型 ,均有染色体t( 8;2 1 )异常。治疗中髓外复发主要在ALL患儿的CNSL ,发病率 1 4 .5%。结论儿童少年AL髓外浸润多见于T -ALL及M2b患儿 ,与预后不良有关。早期发现 ,积极治疗 ,可降低儿童少年AL的CNSL发病率。

Objective Accessing the clinical extramedullary infiltration characteristics do children and adolescents with acute leukemia (AL) to explore the administration and improve the long time survival. Methods Retrospectively analyzed consecutive 497 children and adolescents with acute leukemia who were diagnosed in our hospital from May 1996 to May 2003. Results Of the patients, sixty-three percent suffered with acute lymphoblastic leukemia (ALL) and the other 33% with ANLL. Children ALL, especially T-ALL, often showed mediastina metastasis when the infiltration took place. The incidence of extramedullary infiltration more frequently occurred in high-risk patients than that in standard-risk patients. At the first diagnosis, the extramedullary infiltration of ANLL (mainly in M 2b, M 4 and M 5) was frequently found in central neural system, skin, eyes, and pleura. Unexpectedly, the CNS infiltration was more often found in patients with ANLL with the median age 12 ys, boy preferential and leukocytosis, than that in children with ALL. The most frequent occurrence of extramedullary infiltration was M 2b together with t(8;21). The majority of extramedullary relapse was CNSL in children with ALL with the relapse rate of 14.5%. Conclusion Extramedullary infiltration was major in children with T-ALL or M 2b and indicated poor prognoses. Earlier diagnosis and appropriate therapy maybe reduce the incidence of extramedullary infiltration.