Treatment of Burkitt leukemia/lymphoma in adults
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jonathan W Friedberg, MD
Jonathan W Friedberg, MD
- Professor of Medicine
- James P Wilmot Cancer Center, University of Rochester
- Section Editors
- Andrew Lister, MD, FRCP, FRCPath, FRCR
Andrew Lister, MD, FRCP, FRCPath, FRCR
- Section Editor — Lymphoproliferative Disorders
- Professor of Medical Oncology
- St. Bartholomew's Hospital, London
- Julie R Park, MD
Julie R Park, MD
- Section Editor — Lymphoma; Pediatric Oncology
- Professor of Pediatrics
- University of Washington
Burkitt lymphoma (BL) is a highly aggressive B cell neoplasm characterized by the translocation and deregulation of the c-Myc gene on chromosome 8. Three distinct clinical forms of BL are recognized: endemic, sporadic, and immunodeficiency-associated. Although these differ in their epidemiology, clinical presentation, and genetic features, they are histologically identical and have similar clinical behavior. All three clinical forms are generally treated in a similar fashion.
BL and Burkitt leukemia are classified as different manifestations of the same disease. Diagnostic criteria have evolved to incorporate immunohistochemical, cytogenetic, and molecular diagnostic techniques. As such, many older studies included a heterogeneous patient population that contained many patients that would not be diagnosed with BL by current criteria.
The treatment of BL is presented here. The epidemiology, clinical presentation, and genetic features are discussed separately. (See "Epidemiology, clinical manifestations, pathologic features, and diagnosis of Burkitt lymphoma".)
Oncologic emergencies and treatment-related hematologic toxicities are common in the highly aggressive NHLs. Physicians must always be alert to their potential presence, and be prepared to deal with them urgently and effectively. In particular, patients with BL are at high risk for the development of tumor lysis syndrome (TLS). Prophylactic therapy for and management of TLS is presented in detail separately. (See "Tumor lysis syndrome: Definition, pathogenesis, clinical manifestations, etiology and risk factors" and "Clinical presentation and diagnosis of non-Hodgkin lymphoma", section on 'Oncologic emergencies'.)
The pretreatment evaluation both determines the bulk of disease and provides information about the individual's comorbidities that are likely to have an impact on treatment options. In addition to a history and physical examination, it is our practice to perform the following pretreatment studies in patients with BL:
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- PRETREATMENT EVALUATION
- INITIAL TREATMENT
- Choice of chemotherapy
- - CODOX-M plus IVAC ("Magrath regimen")
- - CALGB 9251
- - HyperCVAD
- - Dose-adjusted EPOCH
- Incorporation of rituximab
- Our approach to treatment
- SPECIAL SCENARIOS
- AIDS-related lymphoma
- Patients with cardiac disease
- Older adults
- CNS involvement
- PATIENT FOLLOW-UP
- Response evaluation
- Surveillance for relapse
- Survivorship issues
- TREATMENT OF RECURRENT OR REFRACTORY DISEASE
- CLINICAL TRIALS
- SUMMARY AND RECOMMENDATIONS