Cancer Res Treat.  2014 Oct;46(4):358-365. 10.4143/crt.2013.047.

Clinical Outcome of Relapsed or Refractory Burkitt Lymphoma and Mature B-Cell Lymphoblastic Leukemia in Children and Adolescents

• Kim H
• Park ES
• Lee SH
• Koo HH
• Kim HS
• Lyu CJ
• Jun SE
• Lim YT
• Baek HJ
• Kook H
• Lee JW
• Kang HJ
• Park KD
• Shin HY
• Ahn HS

• KMID: 2380373
• DOI: http://doi.org/10.4143/crt.2013.047

Abstract

PURPOSE
Despite the rapid improvement in survival rate from Burkitt lymphoma and mature B-cell lymphoblastic leukemia (B-ALL) in children, a small subset of patients do not respond to first-line chemotherapy or experience relapse (RL). Herein, we report the clinical characteristics and outcomes of these patients.
MATERIALS AND METHODS
RL or refractory Burkitt lymphoma and mature B-ALL in 125 patients diagnosed from 1990 to 2009 were retrospectively analyzed.
RESULTS
Nineteen patients experienced RL or progressive disease (PD). Among them, 12 patients had PD or RL less than six months after initial treatment and seven had late RL. Seven patients achieved complete response (CR), 11 had PD, and one had no more therapy. Six patients who achieved CR survived without evidence of disease and four of them underwent high-dose chemotherapy (HDC) followed by stem cell transplantation (SCT). However, 11 patients who failed to obtain CR eventually died of their disease. Five-year overall survival (OS) was 31.6+/-10.7%. OS of patients with late RL was superior to that of patients with early RL (57.1+/-18.7%, vs. 16.7+/-10.8%, p=0.014). Achievement of CR after reinduction had significant OS (p < 0.001). OS for patients who were transplanted was superior (p < 0.01). In multivariate analysis, achievement of CR after reinduction chemotherapy showed an association with improved OS (p=0.05).
CONCLUSION
Late RL and chemotherapy-sensitive patients have the chance to achieve continuous CR using HDC/SCT, whereas patients who are refractory to retrieval therapy have poor prognosis. Therefore, novel salvage strategy is required for improvement of survival for this small set of patients.

Keyword

Burkitt lymphoma; Recurrence; Children

MeSH Terms

Adolescent*
B-Lymphocytes*
Burkitt Lymphoma*
Child*
Drug Therapy
Humans
Multivariate Analysis
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
Prognosis
Recurrence
Retrospective Studies
Stem Cell Transplantation
Survival Rate

Figure

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  Fig. 1. (A) Overall survival of relapsed or primary refractory disease. Median survival was seven months (95% confidence interval [CI], 5.6 to 8.4). (B) Overall survival of patients who achieved complete response (CR) and non-CR to reinduction chemotherapy, which is statistically significant (p < 0.001). Median survival was 5.5 months (95% CI, 3.4 to 6.6) for patients who had non-CR. (C) Overall survival and time to relapse. Median survival was five months for patients who had primary refractory or early relapse. Survival rate for primary refractory or early relapse and late relapse was 16.7±10.8% and 57.1±18.7, respectively (p < 0.05). (D) Overall survival and hematopoietic stem cell transplantation. Median survival was 6.5 months (95% CI, 4.6 to 8.4) for patients who were not transplanted. Overall survival of both groups was statistically significant (p < 0.01).

Reference

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