[Clinical and prognostic analysis of 186 children with Burkitt's lymphoma].

Objective: To analyze the clinical features and prognostic factors of childhood Burkitt's lymphoma and to summarize the therapeutic effect of the mature B-cell lymphoma regimen of Beijing Children's Hospital. Methods: It was a retrospective study. From January 2007 to December 2015, 186 patients below 18 years of age with newly diagnosed, untreated Burkitt's lymphoma were enrolled. Three cases were eliminated because of the abandonment of the treatment and 183 cases were stratified and treated according to the mature B-cell lymphoma regimen of Beijing Children's Hospital, groups were as follows: A, n= 1; B, n= 59; C, n= 123 and 97 patients in group C received combined rituximab therapy during the treatment. The clinical features and therapeutic effects of patients were analyzed, overall survival (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier method. COX regression was used to identify the prognostic factors. Results: The median age at diagnosis was 5 (1-14) years. There were 159 males (85.5%) and 27 females (14.5%) , the male-to-female ratio was 5.9∶1. A total of 174 cases (93.5%) evolved to stage Ⅲ and Ⅳ. Eight cases did not achieve remission and progressed to death, 9 cases relapsed. Only 5 patients (2.7%) died of treatment-related complications. With a median follow-up time of 48.0 (0.5-128.0) months, the 5-year OS rate and EFS rate were (89.1±2.3) % and (87.8±2.5) %. There was significant difference in the 5-year EFS rate between group B and C ( (94.9±2.9) % vs. (84.0±3.4) %, χ(2)=4.258, P =0.039). The 5-year EFS rate was (73.1±8.7) % and (86.7±3.7) % for patients in the group C treated with chemotherapy only and those treated with chemotherapy combined rituximab, but no statistical difference was found between them (χ(2)=3.360, P= 0.067) . Central nervous system (CNS) involvement, insensitivity to early phase chemotherapy, residual diseases in mid-term evaluation were independent unfavorable prognostic factors ( HR =6.167, 9.102, 3.104, 95 %CI : 2.293-16.592, 1.837-45.107, 1.182-8.153) . Conclusions: The large dose, short course treatment of mature B-cell lymphoma regimen of Beijing Children's Hospital is effective for pediatric Burkitt's Lymphoma. Combined treatment with rituximab can improve the efficacy. CNS involvement, insensitivity to early phase chemotherapy, residual diseases in mid-term evaluation are associated with increased risk of poor prognosis.