Clinical Outcome of Relapsed or Refractory Burkitt Lymphoma and Mature B-Cell Lymphoblastic Leukemia in Children and Adolescents
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PurposeDespite the rapid improvement in survival rate from Burkitt lymphoma and mature B-celllymphoblastic leukemia (B-ALL) in children, a small subset of patients do not respond tofirst-line chemotherapy or experience relapse (RL). Herein, we report the clinical characteristicsand outcomes of these patients. Materials and MethodsRL or refractory Burkitt lymphoma and mature B-ALL in 125 patients diagnosed from 1990to 2009 were retrospectively analyzed. ResultsNineteen patients experienced RL or progressive disease (PD). Among them, 12 patientshad PD or RL less than six months after initial treatment and seven had late RL. Sevenpatients achieved complete response (CR), 11 had PD, and one had no more therapy. Sixpatients who achieved CR survived without evidence of disease and four of them underwenthigh-dose chemotherapy (HDC) followed by stem cell transplantation (SCT). However, 11patients 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 earlyRL (57.1±18.7%, vs. 16.7±10.8%, p=0.014). Achievement of CR after reinduction hadsignificant 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 anassociation with improved OS (p=0.05). ConclusionLate RL and chemotherapy-sensitive patients have the chance to achieve continuous CRusing HDC/SCT, whereas patients who are refractory to retrieval therapy have poorprognosis. Therefore, novel salvage strategy is required for improvement of survival for thissmall set of patients.
Abstract
PurposeDespite the rapid improvement in survival rate from Burkitt lymphoma and mature B-celllymphoblastic leukemia (B-ALL) in children, a small subset of patients do not respond tofirst-line chemotherapy or experience relapse (RL). Herein, we report the clinical characteristicsand outcomes of these patients. Materials and MethodsRL or refractory Burkitt lymphoma and mature B-ALL in 125 patients diagnosed from 1990to 2009 were retrospectively analyzed. ResultsNineteen patients experienced RL or progressive disease (PD). Among them, 12 patientshad PD or RL less than six months after initial treatment and seven had late RL. Sevenpatients achieved complete response (CR), 11 had PD, and one had no more therapy. Sixpatients who achieved CR survived without evidence of disease and four of them underwenthigh-dose chemotherapy (HDC) followed by stem cell transplantation (SCT). However, 11patients 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 earlyRL (57.1±18.7%, vs. 16.7±10.8%, p=0.014). Achievement of CR after reinduction hadsignificant 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 anassociation with improved OS (p=0.05). ConclusionLate RL and chemotherapy-sensitive patients have the chance to achieve continuous CRusing HDC/SCT, whereas patients who are refractory to retrieval therapy have poorprognosis. Therefore, novel salvage strategy is required for improvement of survival for thissmall set of patients.
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