Blood Res.  2020 Dec;55(4):262-274. 10.5045/br.2020.2020220.

Long-term treatment outcomes of children and adolescents with lymphoblastic lymphoma treated with various regimens:a single-center analysis

• Choi HJ
• Shin J
• Kang S
• Suh JK
• Kim H
• Koh KN
• Im HJ

• KMID: 2509965
• DOI: http://doi.org/10.5045/br.2020.2020220

Abstract

Background
Lymphoblastic lymphoma (LBL) is the second most common subtype of pediatric non-Hodgkin lymphoma. Modified treatments derived from the LSA2-L2 regimen resulted in encouraging survival, but toxicities and long-term sequelae have been problematic. At present, the acute lymphoblastic leukemia (ALL)-type protocol has demonstrated efficacy in LBL. We analyzed the outcomes of children and adolescents with LBL treated with various regimens.
Methods
From 1991‒2018, this study enrolled 63 patients diagnosed with LBL at Asan Medical Center. Medical records were retrospectively analyzed.
Results
Among 63 patients, most patients (38.1%) presented with stage IV at diagnosis, and two had central nervous system (CNS) involvement. At a median follow-up of 160 months, the 5-year event free survival (EFS), overall survival (OS), and relapse free survival (RFS) were 68.8%, 79.3%, and 71.3%, respectively. Among 61 patients who received chemotherapy, 27 patients (44.3%) received the NY protocol, and 14 (23.0%) received the ALL-type protocol. There was no significant difference in 5-yr OS (85.2%/78.6%), EFS (73.5%/78.6%), and RFS (73.5%/78.6%) between the NY and ALL protocol groups, regardless of immunophenotype. Thirteen patients (21.3%) received prophylactic cranial radiotherapy with no difference in the incidence of CNS relapse based on irradiation.
Conclusion
This study showed no difference in outcome between the NY and ALL-type protocols, regardless of stage or immunophenotype. In addition to improving the effectiveness of treatment, it is necessary to continuously appraise the appropriate chemotherapy regimen, considering toxicities and long-term prognosis, for pediatric LBL.

Keyword

Lymphoblastic lymphoma; Child; Survival; Chemotherapy; New York protocol

Figure

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  Fig. 1 Survival outcomes of patients. The overall survival (A), event-free survival (B), and relapse-free survival rates (C) were 79.3±5.1%, 68.8±6.6%, and 71.3±5.7%, respectively.

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  Fig. 2 When analyzed according to initial stage, patients with stage III/IV disease show lower survival rates than others. However the overall survival (A), and event-free survival rates (B) were not statistically different.

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  Fig. 3 When advanced stage patients were analyzed according to immunophenotype, the overall survival (A) and event-free survival rates (B) were not statistically different between B- and T-LBL (N=43).

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  Fig. 4 There were 18 events, and 13 patients died. All patients alive to date are disease-free.

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  Fig. 5 Treatment outcome according to initial chemotherapy protocol. The overall survival (A), event-free survival (B), and relapse-free survival rates (C) were not statistically different between patients who received the ALL-type and NY regimens (N=41).

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