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15 - B-cell acute lymphoblastic leukemia and Burkitt lymphoma

from Section 3 - Evaluation and treatment

Published online by Cambridge University Press:  05 April 2013

By
John T. Sandlund  and
Mihaela Onciu
Edited by
Ching-Hon Pui
Ching-Hon Pui
Affiliation:
St Jude's Children's Research Hospital
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Summary

Introduction

B-cell acute lymphoblastic leukemia (ALL) accounts for approximately 2% of childhood ALL in the USA. This malignancy has clinical, histologic, immunophenotypic, and cytogenetic features similar to those of Burkitt lymphoma, a high-grade B-cell non-Hodgkin lymphoma. Burkitt lymphoma and B-cell ALL are usually considered to represent a spectrum of the same disease process. In children, the distinction between Burkitt lymphoma with bone marrow involvement and B-cell ALL is arbitrarily based on the degree of bone marrow infiltration. Patients who have less than 25% replacement of normal marrow with lymphoblasts are considered to have advanced (stage IV) Burkitt lymphoma, whereas those with more extensive marrow replacement are assigned the diagnosis of B-cell ALL. These two groups are treated with protocols similar to those used for patients with advanced-stage Burkitt lymphoma that has not disseminated to the marrow (stage III). Although limited-stage Burkitt lymphoma is included in this disease spectrum, it is less common than the advanced forms and is treated with much less intensive therapy. Hence, in describing treatment, we will focus on stages III and IV Burkitt lymphoma and B-cell ALL.

Advances in our understanding of the molecular pathogenesis of these malignancies and improvements in treatment outcome have been striking over the past 25 years. Event-free survival rate can be expected as high as 90% for children with B-cell ALL to date, as opposed to under 50% in the mid 1980s.

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Information
Childhood Leukemias , pp. 383 - 394
Publisher: Cambridge University Press
Print publication year: 2012

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