Understanding Lymphoma

Burkitt Lymphoma

Burkitt lymphoma (BL) is a rare but highly aggressive (fast-growing) form of mature B-cell non-Hodgkin
lymphoma (NHL). The disease typically involves younger patients and represents the most common type
of pediatric NHL. It also may be seen in elderly patients as well. It may affect the jaw, bones of the face, bowel, kidneys,
ovaries, and in some cases, it may spread to the central nervous system (CNS, i.e., brain and spinal cord). At diagnosis, a
sample of cerebrospinal fluid may be taken to determine if the disease has spread to the CNS.
Patients with BL may experience tumor lysis syndrome, which occurs when tumor cells release their contents into
the bloodstream. Symptoms may include nausea and vomiting, shortness of breath, irregular heartbeat, clouding
of the urine, lethargy, or joint discomfort. This condition is potentially severe and can occur spontaneously or after
chemotherapy. Tumor lysis syndrome can cause kidney and heart damage, seizures, loss of muscle control, and in some
cases, death. However, this condition can be managed with increased fluids and supportive medications like allopurinol
(Aloprim, Lopurin, Zyloprim) or rasburicase (Elitek).
There are three main types of BL: endemic, sporadic, and immunodeficiency-associated.
• Endemic BL typically affects boys between the ages of 4 and 7 years in specific geographic areas (Equatorial Africa,
Papua New Guinea, and regions of South America), where it is the most common childhood cancer. Endemic BL is
linked to infection with Epstein-Barr virus (EBV) and is rare outside these specific areas.
• Sporadic BL occurs in children and adults worldwide. It accounts for about one to two percent of NHLs in adults and
is one of the most common types of childhood lymphoma in the US.
• The immunodeficiency-related variety of BL is most common in people with human immunodeficiency virus/acquired
immunodeficiency syndrome (HIV/AIDS). Immunodeficiency-related BL can also occur in patients who have inherited
immune deficiencies or who take immunosuppressive medications to prevent rejection after organ transplant.
A genetic alteration (called translocation) of the MYC gene in the lymphoma cells (not the genes that you inherit) is a
hallmark of BL, making this an important finding for diagnosis of the disease. In adults, BL is sometimes difficult to
distinguish from diffuse large B-cell lymphoma (DLBCL)—a more common form of aggressive mature B-cell NHL.
Accurately distinguishing between BL and DLBCL is critical, because each disease is treated differently.

TREATMENT OPTIONS The Dose-Adjusted EPOCH (DA EPOCH-R) regimen includes:

• Etoposide (Etopophos, Toposar, VePesid), prednisone,
Because BL is extremely aggressive, diagnosis is frequently a vincristine (Oncovin, Vincasar), cyclophosphamide, and
medical emergency, requiring urgent hospitalization and rapid doxorubicin plus rituximab (Rituxan)
treatment. However, BL is often very responsive to intensive
• Intrathecal methotrexate for patients who are at low risk
combination chemotherapy regimens and cure rates are high.
and without CNS involvement, or high- risk patients who are
The choice of initial therapy depends on different factors, such not able to tolerate more aggressive treatments
as age, the presence of other medical conditions, stage, and risk
level of BL. Standard of care treatment typically involves short The HyperCVAD (R+HyperCVAD) regimen includes:
courses of aggressive chemotherapy regimens in combination • Cyclophosphamide, vincristine, doxorubicin, and
with rituximab (Rituxan). Less intensive regimens might be used dexamethasone alternating with high-dose methotrexate
for patients with low-risk BL or who are not fit for intensive and cytarabine (Cytosar) plus rituximab
chemotherapy. Specific treatment options for adults include the
following regimens: • Intrathecal therapy may be given for a longer duration than
the other treatments listed herein

Understanding Lymphoma Series Helpline: 800 500 9976 | [email protected]

The CODOX-M regimen (original or modified) consists of: • Sepantronium bromide (PC-002)
• Cyclophosphamide, doxorubicin, and vincristine with • Clofarabine (Clolar, Evoltra) and mitoxantrone (Novantrone)
intrathecal methotrexate and cytarabine, followed by • Vorinostat (Zolinza) with chemotherapy
high-dose systemic methotrexate with or without
rituximab, for three cycles
• This regimen is sometimes alternated with IVAC
(ifosfamide, intrathecal methotrexate, etoposide, and
CLINICAL TRIALS
high-dose cytarabine)
Clinical trials are crucial in identifying effective drugs and
The CALGB (R+CALGB) regimen includes: determining optimal doses for patients with lymphoma.
Patients interested in participating in a clinical trial should
• Cyclophosphamide, prednisone, ifosfamide, methotrexate, view the Understanding Clinical Trials fact sheet on LRF’s
vincristine, cytarabine, etoposide, doxorubicine, and website (visit lymphoma.org/publications), and the Clinical
dexamethasone Trials Search Request Form at lymphoma.org, talk to their
• Outcomes improved when rituximab (Rituxan) was added physician, or contact the LRF Helpline for an individualized
to the above regimen clinical trial search by calling (800) 500-9976 or emailing
[email protected].
The LMB (R+LMB) regimen includes:
• Cyclophosphamide, doxorubicin, vincristine, and prednisone
• Intermediate or high-risk groups may additionally receive FOLLOW-UP
regimens including cytarabine, methotrexate, and etoposide
Patients with lymphoma should have regular visits with a
CNS involvement is the strongest risk factor for relapse
physician familiar with their medical history and the treatments
(disease returns after treatment). Patients with BL without CNS
they have received. Medical tests (such as blood tests and
involvement require prophylaxis (preventive treatment). The
computed tomography [CT] scans) may be required at various
frequency of these treatments, which are given intrathecally
times during remission (disappearance of signs and symptoms)
(injected into the spinal fluid), depends on whether or not CNS
to evaluate the need for additional treatment.
involvement is present at diagnosis.
Patients and their caregivers are encouraged to keep copies of
HIV-related BL should be treated with similar regimens as for
all medical records and test results as well as information on
HIV-negative patients with BL. Antiretroviral therapy can be
the types, amounts, and duration of all treatments received.
safely administered with chemotherapy. Different combination
This documentation will be important for keeping track of
chemotherapy regimens are used to treat BL in children and
any side effects resulting from treatment or potential disease
adolescents, and younger patients tend to have both excellent
recurrences.
chemotherapy responses and particularly high cure rates. For
this reason, the current trend in the treatment of children is
focused on decreasing toxicity by reducing the overall amount of
chemotherapy used to treat the disease. LRF’S HELPLINE AND LYMPHOMA
SUPPORT NETWORK

TREATMENTS UNDER INVESTIGATION A lymphoma diagnosis often triggers a range of feelings and
concerns. In addition, cancer treatment can cause physical
Ongoing clinical trials are investigating various combination discomfort. The LRF Helpline staff members are available to
therapy regimens, including new agents combined with the answer your general questions about a lymphoma diagnosis
agents mentioned above. Participation in a clinical trial is and treatment information, as well as provide individual support
highly encouraged when available. New agents are also being and referrals to you and your loved ones. Callers may request
investigated alone or as part of combination therapy in relapsed the services of a language interpreter. A part of the Helpline is
or refractory (does not respond to treatment) disease, including LRF’s one-to-one peer support programs, Lymphoma Support
the following: Network. This program connects patients and caregivers with
volunteers who have experience with BL, similar treatments, or
• Tisagenlecleucel (Kymriah) challenges, for mutual emotional support and encouragement.
• Devimistat (CPI-613) Patients and loved ones may find this useful whether the patient
is newly diagnosed, in treatment, or in remission.
• Nivolumab (Opdivo) with lenalidomide (Revlimid)
• Ofatumumab (Arzerra) with DA EPOCH
• Obinutuzumab (Gazyva) with ICE (ifosfamide, carboplatin
and etoposide)
• Venetoclax (Venclexta) with ibrutinib (Imbruvica),
prednisone, obinutuzmab (Gazyva) and lenalomide
(Revlimid)
• Acalabrutinib (Calquence) with pembrolizumab (Keytruda)

Understanding Lymphoma Series Helpline: 800 500 9976 | [email protected]

 MOBILE APP
Focus On Lymphoma is the first mobile application (app) that Resources
provides patients and caregivers comprehensive content based LRF offers a wide range of free resources that address
on their lymphoma subtype, including BL, and tools to help treatment options, the latest research advances, and
manage their lymphoma such as, keep track of medications ways to cope with all aspects of lymphoma and BL. LRF
and blood work, track symptoms, and document treatment side also provides many educational activities, including
effects. The Focus On Lymphoma mobile app is available for our in- person meetings and webinars for people with
download for iOS and Android devices in the Apple App Store and lymphoma. For more information about any of these
Google Play. To learn more about any of these resources, visit resources, visit our websites at lymphoma.org/burkitt
our website at lymphoma.org, or contact the LRF Helpline at or lymphoma.org, or contact the LRF Helpline at
(800) 500-9976 or [email protected]. (800) 500-9976 or [email protected].

LRF appreciates the expertise and John Allan Manali Kamdar, MD Contact LRF:
review of our Editorial Committee: Weill Cornell Medicine University of Colorado
Helpline: (800) 500-9976
Leo I. Gordon, MD, FACP Jennifer E. Amengual, MD Peter Martin, MD,
Columbia University Weill Cornell Medicine Email: [email protected]
Co-Chair
Robert H. Lurie Comprehensive Cancer Jonathon Cohen Anthony Mato, MD www.lymphoma.org
Center of Northwestern University Emory University School of Medicine Memorial Sloan Kettering Cancer Center
Kristie A. Blum, MD Alex Herrera, MD Neha Mehta-Shah, MD, MSCI
Co-Chair City of Hope Washington University School
Emory University School of Medicine Shana Jacobs, MD of Medicine in St. Louis
Children’s National Hospital Pierluigi Porcu, MD
Thomas Jefferson University

Supported through grants from:

Understanding Lymphoma fact sheet series is published by the Lymphoma Research Foundation (LRF) for the purpose of informing and educating readers. Facts and statistics
were obtained using published information, including data from the Surveillance, Epidemiology, and End Results (SEER) Program. Because each person’s body and response
to treatment is different, no individual should self-diagnose or embark upon any course of medical treatment without first consulting with his or her physician. The medical
reviewer, the medical reviewer’s institution, and LRF are not responsible for the medical care or treatment of any individual.
© 2022 Lymphoma Research Foundation

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