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Blood, 15 November 2004, Vol. 104, No. 10, pp. 3009-3020.
Prepublished online as a Blood First Edition Paper on July 20, 2004; DOI 10.1182/blood-2004-02-0405.
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Adult Burkitt leukemia and lymphoma
Kristie A. Blum,
Gerard Lozanski, and
John C. Byrd
From the Division of Hematology and Oncology, The Ohio State University, Columbus; the Division of Pathology, The Ohio State University, Columbus; and the Division of Medicinal Chemistry, Department of Pharmacy, The Ohio State University, Columbus.
The World Health Organization Classification of Lymphoid Neoplasms identifies Burkitt lymphoma/leukemia as a highly aggressive mature B-cell neoplasm consisting of endemic, sporadic, and immunodeficiency-associated variants. These subtypes share many morphologic and immunophenotypic features, but differences exist in their clinical and geographic presentations. All of these subtypes possess chromosomal rearrangements of the c-myc oncogene, the genetic hallmark of Burkitt lymphoma that contributes to lymphomagenesis through alterations in cell cycle regulation, cellular differentiation, apoptosis, cellular adhesion, and metabolism. Brief-duration, high-intensity chemotherapy regimens containing aggressive central nervous system prophylaxis have had remarkable success in the treatment of this disease, with complete remission rates of 75% to 90% and overall survivals reaching 50% to 70% in adults. Although Burkitt lymphoma cells are extremely chemosensitive, biologically targeted therapies should be developed because current treatment options are suboptimal for patients with poor prognostic features or in the setting of relapsed disease.
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