Skip to main content Accessibility help
×
Hostname: page-component-7c8c6479df-995ml Total loading time: 0 Render date: 2024-03-29T05:12:34.985Z Has data issue: false hasContentIssue false

11 - Burkitt and lymphoblastic lymphoma: clinical therapy and outcome

Published online by Cambridge University Press:  18 December 2013

Nirali N. Shah
Affiliation:
National Institutes of Health
Alan S. Wayne
Affiliation:
University of Southern California
Wyndham H. Wilson
Affiliation:
National Institutes of Health
Robert Marcus
Affiliation:
King's College London
John W. Sweetenham
Affiliation:
University of Utah
Michael E. Williams
Affiliation:
University of Virginia School of Medicine, Charlottesville
Get access

Summary

Introduction and presentation

Burkitt (BL) and lymphoblastic lymphomas (LBL) are highly aggressive diseases with distinct natural histories and clinical presentations. BL mostly occurs in the first two decades of life and accounts for 1–2% of all lymphomas. Three clinical variants are recognized: (1) endemic BL, which is primarily found in equatorial Africa; (2) sporadic BL, which presents worldwide but is the most common type in western countries; and (3) immunodeficiency-associated BL, which is associated with HIV infection. There are important clinical differences in these variants (Table 11.1), with endemic BL involving the jaw, orbit, and paraspinal regions in half of the cases as well as the mesentery and gonads, while sporadic BL mostly involves the distal ileum, cecum, and/or mesentery, and rarely the jaw. When bulky or disseminated disease is present, extranodal involvement of the ovaries, kidney, breasts, and/or CNS may be seen. Clinical presentation in a Berlin–Frankfurt–Munster Group (BFM) series of 152 pediatric patients included advanced stage (III/IV) disease in 38%, bone marrow involvement in 33%, and central nervous system (CNS) disease in 4%. Of the patients in this series, 27% presented as acute leukemia, referred to as the L3 subtype of acute lymphoblastic leukemia (ALL) within the French-American-British (FAB) classification, or B-ALL. BL infrequently presents in adults, but does occur with increased frequency in patients with HIV infection.

LBL is most commonly a malignancy of T-cell precursor cells, and, as such, it is identical to T-cell acute lymphoblastic leukemia (T-ALL).

Type
Chapter
Information
Lymphoma
Pathology, Diagnosis, and Treatment
, pp. 172 - 190
Publisher: Cambridge University Press
Print publication year: 2013

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×