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26 - Burkitt lymphoma

Published online by Cambridge University Press:  10 January 2011

By
Julie M. Vose
Edited by
Susan O'Brien ,
Julie M. Vose  and
Hagop M. Kantarjian
Julie M. Vose
Affiliation:
Section of Hematology/Oncology, Nebraska Medical Center, Omaha, NE, USA
Susan O'Brien
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
Julie M. Vose
Affiliation:
University of Nebraska Medical Center, Omaha
Hagop M. Kantarjian
Affiliation:
University of Texas/MD Anderson Cancer Center, Houston
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Summary

Introduction

Burkitt lymphoma (BL) is an uncommon B-cell lymphoma characterized by high proliferation rate and cytogenetic changes related to c-Myc proto-oncogene overexpression. It encompasses a heterogeneous group of highly aggressive B-cell lymphoma. It is seen frequently in children and young adults in the endemic areas. As the result of the world epidemic of AIDS, the number of adult BL cases has increased significantly in the last few decades. In the United States, BL has an incidence of 1200 patients per year.

This lymphoma was first described by Denis Burkitt in 1958 in children in Africa. This type of endemic BL was described in children with jaw tumors with eventual spread to extranodal sites, particularly the bone marrow and leptomeninges. In the USA, BL is more commonly diagnosed in adult patients over age 40 years. According to the Surveillence, Epidemiology and End Results (SEER) registry, 59% of the BL cases in the USA are in patients over the age of 40 years.

The challenge of correctly identifying the BL in an individual patient becomes particularly critical as BL is rapidly fatal if not treated rapidly and appropriately with brief intensive chemotherapy. Initiation of the correct treatment early has yielded excellent results; however, when patients with BL were treated with regimens for diffuse large B-cell lymphoma (DLBCL), the long-term outcomes were inferior. On the other hand, the intensive therapy is not the favored choice for DLBCL, since it is associated with higher toxicity.

Type
Chapter
Information
Management of Hematologic Malignancies , pp. 494 - 502
Publisher: Cambridge University Press
Print publication year: 2010

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