Background:With current chemotherapy treatment,>90%of survival has been obtained for Burkitt lymphoma(BL).In this study,the demographic characteristics and treatment outcomes are presented for 78 children in China ...Background:With current chemotherapy treatment,>90%of survival has been obtained for Burkitt lymphoma(BL).In this study,the demographic characteristics and treatment outcomes are presented for 78 children in China with central nervous system-positive(CNS+)BL.Methods:This retrospective study consecutively enrolled 78 CNS+BL patients in Beijing Children’s Hospital(BCH)from 2007 to 2019 who received the BCH B-cell non-Hodgkin’s lymphoma regimen(modified by French-American-British mature lymphoma B-cell 96[FAB/LMB96]C1 arm±rituximab).Clinical characteristics,methods of disease detection in the CNS,and outcomes were evaluated.Univariate and multivariate analyses were used to assess prognostic factors.Results:The median age of 65 boys and 13 girls at the time of diagnosis was 5.7 years(ranging from 1 to 14 years).Patients were followed up for a median time of 34 months(ranging from 1 to 72 months).Bone marrow invasion was found in 38(48.7%)patients.There were 48(61.5%),44(56.4%),and 25(32%)patients with cranial nerve palsy,intracerebral mass(ICM),and parameningeal extension,respectively.Abnormal cerebrospinal fluid(CSF)morphology and CSF immunophenotype appeared in 15(19.2%)and 15(19.2%)patients,respectively.There were 69(88.5%)patients treated with chemotherapy combined with rituximab,and nine patients were treated solely with chemotherapy.Finally,five patients died of treatment-related infection,recurrence occurred for 13,and one developed a second tumor.The 3-year overall survival and event-free survival rates were 78.9%±4.7%and 71.4%±6.0%,respectively.Treatment with chemotherapy only,ICM positivity,and>4 organs involved at diagnosis were independent risk factors.Conclusions:Rituximab combined with a modified LMB96 regimen has greatly increased the efficacy of treatment for Chinese children with CNS+BL,and with the continuous collection of outcome data,treatment-related complications are decreasing.For further verification,a large sample multicentre randomized controlled study should be performed to explore a treatment scheme for Chinese children with even greater efficacy.展开更多
基金by a grant from the special fund of the pediatric medical coordinated development center of the Beijing Children’s Hospital(No.XTZD20180204).
文摘Background:With current chemotherapy treatment,>90%of survival has been obtained for Burkitt lymphoma(BL).In this study,the demographic characteristics and treatment outcomes are presented for 78 children in China with central nervous system-positive(CNS+)BL.Methods:This retrospective study consecutively enrolled 78 CNS+BL patients in Beijing Children’s Hospital(BCH)from 2007 to 2019 who received the BCH B-cell non-Hodgkin’s lymphoma regimen(modified by French-American-British mature lymphoma B-cell 96[FAB/LMB96]C1 arm±rituximab).Clinical characteristics,methods of disease detection in the CNS,and outcomes were evaluated.Univariate and multivariate analyses were used to assess prognostic factors.Results:The median age of 65 boys and 13 girls at the time of diagnosis was 5.7 years(ranging from 1 to 14 years).Patients were followed up for a median time of 34 months(ranging from 1 to 72 months).Bone marrow invasion was found in 38(48.7%)patients.There were 48(61.5%),44(56.4%),and 25(32%)patients with cranial nerve palsy,intracerebral mass(ICM),and parameningeal extension,respectively.Abnormal cerebrospinal fluid(CSF)morphology and CSF immunophenotype appeared in 15(19.2%)and 15(19.2%)patients,respectively.There were 69(88.5%)patients treated with chemotherapy combined with rituximab,and nine patients were treated solely with chemotherapy.Finally,five patients died of treatment-related infection,recurrence occurred for 13,and one developed a second tumor.The 3-year overall survival and event-free survival rates were 78.9%±4.7%and 71.4%±6.0%,respectively.Treatment with chemotherapy only,ICM positivity,and>4 organs involved at diagnosis were independent risk factors.Conclusions:Rituximab combined with a modified LMB96 regimen has greatly increased the efficacy of treatment for Chinese children with CNS+BL,and with the continuous collection of outcome data,treatment-related complications are decreasing.For further verification,a large sample multicentre randomized controlled study should be performed to explore a treatment scheme for Chinese children with even greater efficacy.
