Burkitt Lymphoma and Burkitt-like Lymphoma Guidelines

Updated: Dec 20, 2019
  • Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Guidelines

Guidelines Summary

Guidelines contributors:  Priyank P Patel, MD , Hematology/Oncology Fellow, Roswell Park Cancer Institute, University at Buffalo;  Francisco J Hernandez-Ilizaliturri, MD; Chief, Lymphoma and Myeloma Section; Professor of Medicine, Department of Medical Oncology; Director of The Lymphoma Translational Research Program; Associate Professor of Immunology, Roswell Park Cancer Institute 

Non-Hodgkin Lymphoma (NHL)  Classification Schemas

The three most commonly used classification schemas for non-Hodgkin lymphoma (NHL) are as follows:

  • National Cancer Institute’s Working Formulation (IWF) [71]

  • Revised European-American Classification of Lymphoid Neoplasms (REAL) [72]

  • World Health Organization (WHO) classification [2]

The Working Formulation, originally proposed in 1982, classified and grouped lymphomas by morphology and clinical behavior (ie, low, intermediate, or high grade) with 10 subgroups labeled A to J. [71] In 1994, the Revised European-American Lymphoma (REAL) classification attempted to apply immunophenotypic and genetic features in identifying distinct clinicopathologic NHL entities. [72]

The World Health Organization (WHO) classification, first introduced in 2001 and updated in 2008 and 2016, further elaborates upon the REAL approach. This classification divides NHL into two groups: those of B-cell origin and those of T-cell/natural killer (NK)–cell origin. [25, 2]

Although considered obsolete, the National Cancer Institute’s Working Formulation (IWF) classification is still used mainly for historical data comparisons. [71]

World Health Organization classification

The 2008 WHO modification of the REAL classification of NHL is based on morphology and cell lineage. The WHO classification identifies the following three clinical variants of Burkitt lymphoma (BL) [25] :

  • Endemic (eBL) – The most common form of childhood malignancy in equatorial Africa, associated with Epstein-Barr virus (EBV) infection

  • Sporadic (sBL) – The majority of cases are in the United States and Europe; up to 30% are associated with EBV

  • Immunodeficiency associated – Occurs in patients with HIV infection, post-transplantation immunosuppression, and congenital immunodeficiency

The 2016 revision of the WHO classification recognizes the following two molecular entities [2] :

  • Burkitt lymphoma - TCF3 or ID3 mutations in up to about 70% of cases
  • Burkitt-like lymphoma with 11q aberration - New provisional entity that closely resembles Burkitt lymphoma but lacks MYC rearrangement and has some other distinctive features
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Diagnosis

In addition to its general guidance on diagnosis of lymphoma, the National Comprehensive Cancer Network (NCCN) recommends the following studies to establish a diagnosis of Burkitt lymphoma [73] :

  • Immunohistochemistry panel: CD45(LCA), CD20, CD3, BCL2, BCL6, Ki-67, TdT or

  • Cell surface marker analysis by flow cytometry: kappa/lambda, CD45, CD20, CD3, CD5, CD19, CD10, TdT

  • Fluorescence in situ hybridization (FISH) or cytogenetics for detection of t(8;14), MYC.

  • Epstein-Barr encoding region in situ hybridization (EBER-ISH) can be used to identify EBV

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Risk stratification

A prognostic scoring system was developed in 2013 using the Surveillance, Epidemiology, and End Results (SEER) database. Risk factors and points assigned are as follows [74] :

  • Age 40-59 years or black race/ethnicity: 1 point

  • Age 60-79 years or stage III/IV disease: 2 points

  • Age 80 years and older: 4 points

The four risk groups based on the scoring system are as follows:

  • Low risk (0-1 point)

  • Low-intermediate risk (2 points)

  • High-intermediate risk (3 points)

  • High risk (≥4 points)

With this model, relative survival rates at 5 years are as follows:

  • Low risk - 71%

  • Low-intermediate - 55%

  • High-intermediate - 41%

  • High-risk - 29%

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Treatment

Because of the complexity of the disease, NCCN guidelines recommend that treatment of Burkitt lymphoma be given at centers with expertise in the management of the disease. Recommended chemotherapy regimens include the following [73] :

  • Cancer and Leukemia Group B (CALGB) 10002 regimen

  • CODOX-M/IVAC (cyclophosphamide, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, high-dose cytarabine) – Original or modified, with or without addition of rituximab

  • Dose-adjusted EPOCH (etoposide, prednisone, vincristine [Oncovin], cyclophosphamide, doxorubicin [hydroxydaunorubicin]), with rituximab (DA-EPOCH-R)

  • Hyper-CVAD (cyclophosphamide, vincristine, doxorubicin [Adriamycin], dexamethasone alternating with high-dose methotrexate and cytarabine) with rituximab (R-hyper-CVAD)

Other treatment recommendations are as follows [73] :

  • Enrollment in available clinical trials for all patients

  • CHOP is not considered adequate therapy

  • Central nervous system prophylaxis with systemic and/or intrathecal chemotherapy with methotrexate and/or cytarabine

  • Prophylaxis for tumor lysis syndrome is mandatory

Follow-up

The NCCN recommends follow up every 2-3 months for the first year after complete response, then every 3 months for the next year, and every 6 months thereafter. Relapse is rare after 2 years. [73]

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