儿童伯基特和伯基特样淋巴瘤40例的临床研究

目的 认识儿童伯基特淋巴瘤(BL)和伯基特样淋巴瘤(BLL)的病理及临床特点,总结大剂量、短疗程化疗方案的疗效,探讨治疗相关的合并症及预后影响因素.方法 2003年2月-2006年4月住院治疗的淋巴瘤患儿40例,全部行活检病理形态及免疫组化检查,并按WHO新的病理分型标准进行分型,参照儿童非霍奇金淋巴瘤(NHL)的St.Jude分期标准进行分期.采用大剂量、短疗程的改良LMB89方案进行治疗.根据不同分期、不同危险因素及对治疗反应的评估结果,将患儿分成A、B、c三个治疗组.结果 临床特点:发病年龄2～14岁,中位发病年龄6.9岁;男33例,女7例,男:女=4.7:1;腹腔肿物及急腹症为最常见表现.病理示BL 30例、BLL 10例;7例(17.5%)在瘤组织中检测到EBER-1阳性细胞,19例血清中EB病毒抗体阳性.确诊时I～Ⅱ期患儿9例,Ⅲ～Ⅵ期患儿31例;伴中枢侵犯者3例,骨髓侵犯者2例.疗程2～8个月.随访率100%,中位追踪时间22.6个月.至2006年12月底,实际存活35例,1年生存率88.7%,预计3年无进展生存率81.8%.化疗中Ⅲ～Ⅳ度骨髓抑制的发生率在B组和C组分别为97.5%和100%,3例出现重症黏膜炎.5例死亡,其中2例为副作用相关死亡.单因素分析显示,临床分期为Ⅳ期、治疗3个月时评估有残留病灶、病程小于15 d与预后不良相关(P＜0.05).结论 采用大剂量、短疗程方案治疗BL和BLL疗效好,预计3年无进展生存率81.8%.病史小于15 d、诊断时为Ⅳ期、治疗3个月未达完全缓解者与预后不良相关.

Clinical study of 40 children with Burkitt's and Burkitt-like lymphoma

Objective To summarize the histological and clinical characteristics of 40 cases with Burkitt's and Burkitt-like lymphoma in children,to evaluate the effects of treatment with international regimen,and to explore the treatment-related complications and prognostic factors.Methods Forty patients with Burkitt's and Burkitt-like lymphoma were registered in Beijing Children Hospital from Feb 2003 to Apr 2006.The diagnosis was confirmed by histology and immunohistochemistry of biopsy,and clinical staging by the examination of imaging,cerebrospinal fluid and bone marrow based on St.Jude system.Intensive,shortterm chemotherapy witch was modified from LMB89 protocol was given to the patients.Results Of the 40 patients,30 were diagnosed as Burkitt's lymphoma (BL) and 10 as Burkitt-like lymphoma (BLL).Antibody against Epstein-Barr virus (EBV-Ab) was positive in 19 cases at diagnosis,only 7 of the patients were positive for EBER.Thirty-three of the cases were male and 7 female(M:F=4.7:1);the median age was 6 years 9 months.The most frequently seen clinical characteristics were abdominal masses and surgical abdomen.Nine cases were at stage Ⅰ-Ⅱ and 31 cases at stage Ⅲ-Ⅳ at diagnosis:CNS was involved in 4 cases and bone marrow in 2 cases.The courses of treatment were approximately 2-8 months.All the patients were followed up,the median follow-up period was 22.6 months.After chemotherapy,35 patients (88.7%)were still alive during the one-year follow-up.The 3-year event-free survival (EFS) rate was 81.8%.Major toxicity was myelosuppression and mucositis.Stage Ⅲ to Ⅳ of myelosuppression occurred in the most patients with unresected tumor and CNS-involvement.Of 5 patients who died,2 died of infection,2 died of lymphoma progression during chemotherapy.and 1 died of relapse.Conclusion Burkitt's and Burkitt-like lymphoma are the most common NHL in children with rapid clinical process.Outcome was greatly improved by current intensive,short-term chemotherapy regimen,the 3-year EFS was 81.8% including the patients who were in advanced stage.Childhood lymphoma with short clinical history,stage Ⅳ and residual disease after 3 months of therapy are associated with poor prognosis.