Treatment outcome in children with central nervous system-positive Burkitt lymphoma using only intrathecal and systemic chemotherapy combined with rituximab.
10.1097/CM9.0000000000001386
- VernacularTitle:Treatment outcome in children with central nervous system-positive Burkitt lymphoma using only intrathecal and systemic chemotherapy combined with rituximab
- Author:
Shuang HUANG
1
;
Lin JIN
1
;
Jing YANG
1
;
Yan-Long DUAN
1
;
Meng ZHANG
1
;
Chun-Ju ZHOU
2
;
Yong-Hong ZHANG
1
Author Information
1. Beijing Key Laboratory of Pediatric Haematology Oncology, National Discipline of Pediatrics, Ministry of Education, MOE Key Laboratory of Major Diseases in Children, Haematology Oncology Center, Beijing Children's Hospital, Beijing 100045, China.
2. Pathology Department, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
- Publication Type:Journal Article
- MeSH:
Adolescent;
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*;
Burkitt Lymphoma/drug therapy*;
Central Nervous System;
Child;
Child, Preschool;
China;
Cyclophosphamide/therapeutic use*;
Disease-Free Survival;
Female;
Humans;
Infant;
Male;
Neoplasm Recurrence, Local;
Retrospective Studies;
Rituximab/therapeutic use*;
Treatment Outcome
- From:
Chinese Medical Journal
2021;134(11):1329-1334
- CountryChina
- Language:English
-
Abstract:
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 para-meningeal 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.
