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18 - B-cell acute lymphoblastic leukemia and Burkitt lymphoma

from Part III - Evaluation and treatment

Published online by Cambridge University Press:  01 July 2010

By
John T. Sandlund  and
Ian T. Magrath
Edited by
Ching-Hon Pui
John T. Sandlund
Affiliation:
Member, Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA
Ian T. Magrath
Affiliation:
Professor of Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, MD
Ching-Hon Pui
Affiliation:
St. Jude Children's Research Hospital, Memphis
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Summary

Introduction

B-cell acute lymphoblastic leukemia (B-cell ALL) has historically accounted for approximately 2% of childhood ALLs diagnosed in the United States each year. This malignancy has clinical, histologic, immunophenotypic, and cytogenetic features similar to those of Burkitt lymphoma, a high-grade B-cell non-Hodgkin lymphoma (NHL). Hence, B-cell ALL and Burkitt lymphoma 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 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 given the diagnosis of B-cell ALL. These two groups are treated with the same protocols used for patients with advanced-stage Burkitt lymphoma that has not disseminated to the marrow (stage III). Although limited-stage Burkitt lymphoma is also thought to be included in this disease spectrum, it is less common in children than are 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 20 years. Currently, a 75% to 85% event-free survival rate can be expected for children with B-cell ALL. By contrast, less than 50% of these patients were event-free survivors 20 years ago.

Type
Chapter
Information
Childhood Leukemias , pp. 487 - 498
Publisher: Cambridge University Press
Print publication year: 2006

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