目的 了解儿童淋巴母细胞淋巴瘤(LBL)合并中枢神经系统(CNS)侵犯的临床特点,探讨CNS侵犯危险因素,总结应用BCH-LBL-2003方案的疗效.方法 对2003年1月-2009年12月间北京儿童医院血液病中心收治的112例LBL住院患儿中合并CNS侵犯的19例患儿的临床资料进行总结和分析.依据不同危险因素将患儿CNS状态分为3级并采用不同程度的治疗方案.结果 19例患儿中15例为初诊时已发生CNS侵犯,4例为化疗中CNS复发.除l例为临床Ⅲ期外,其余均为Ⅳ期.本组脑膜侵犯10例(51％),脑实质侵犯4例(21％),脊旁浸润3例(16％),颅神经侵犯1例(5％),混合性浸润(脑膜+颅神经)1例(5％).初诊时存在CNS侵犯与预后无明显相关性(P =0.075).头面部侵犯和血清LDH ≥500 IU/L为CNS侵犯危险因素(P＜0.05).结论 儿童LBL易合并CNS侵犯.头面部侵犯和血清LDH ≥500 IU/L为CNS侵犯危险因素.对CNS侵犯及存在危险因素患儿加强CNS定向治疗可以改善预后.%Objective To explore the clinical characteristics,risk factors of childhood lymphoblastic lymphoma (LBL) combined with central nervous system (CNS) involvement,and evaluate the therapeutic effects of BCH-LBL-2003 regimen modified according to the BFM-90 protocol.Methods There were total 112 children with LBL treated in the Hematology Oncology Center of Beijing Children's Hospital from January 2003 to December 2009,19 of them were combined with CNS involvement.Their clinical data were summarized and analyzed.The patients were classified into 3 groups based on the severity of CNS involvement,and their treatments were stratified accordingly.Results Among 19 cases,15 had CNS involvement at diagnosis,and the other 4 cases got CNS relapse during the chemotherapy.Four cases were in the stage Ⅳ except 1 patient in the stage Ⅲ.Of all the patients,10 cases (53％) had meninges invasion; 4 (21％) had brain parenchyma; 3 (16％) had spinal cord compression; 1 (5％) had simple cranial nerve involvements and 1 (5％) had both meninges and cranial nerve involvements.The CNS involvement at diagnosis had no correlation with the prognosis (P =0.075).The risk factors of CNS involvement included craniofacial invasion (P =0.000) and elevated LDH level more than 500 IU/L (P =0.004).Conclusion Pediatric LBL is easier to be involved with CNS disease.The craniofacial invasion and elevated serum LDH level are the main risk factors of CNS involvement.Intensive CNS-directed therapy is necessary to improve the prognosis for the patients with CNS involvement or with risk factors.
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