• Burkitt Lymphoma

    Burkitt lymphoma is a cancer of mature B-lymphocytes. It is a form of non-Hodgkin lymphoma, which is a type of cancer that originates in cells of the immune system, called lymphocytes.

    Burkitt lymphoma:

    • is the most common type of non-Hodgkin lymphoma in children.
    • often grows first in the lymph tissue of the head and neck, including the tonsils, or in the abdomen.
    • grows rapidly and can spread to many parts of the body, including to the spinal fluid and bone marrow.
    • is called Burkitt leukemia when it presents as tumor cells in the bone marrow and the bloodstream.
    • grows rapidly, so symptoms often progress very quickly and children can become quite sick within a few days to weeks.

    Starting Burkitt lymphoma treatment promptly is important. With current therapies, more than 90% of children with Burkitt lymphoma and leukemia are cured of the disease.

    Burkitt Lymphoma Treatment at Dana-Farber/Boston Children's

    Children with non-Hodgkin lymphoma are treated at Dana-Farber/Boston Children's through the Lymphoma Treatment Program in our Hematologic Malignancies Center. One of the top pediatric cancer centers worldwide, Dana-Farber/Boston Children’s combines the expertise of a premier cancer center – Dana-Farber Cancer Institute – and a world-class children’s hospital – Boston Children’s Hospital – to provide internationally-renowned care for children with cancers of the blood and immune system.

    How is Burkitt lymphoma/leukemia classified?

    Under a microscope, Burkitt lymphoma often has a pattern that looks like a “starry sky” with many small round blue cells interspersed with star-like macrophage cells. Special stains to look at the proteins on the Burkitt lymphoma cells show these cells express a marker called CD20. Specialized tests looking at the chromosomes in these cells often show a rearrangement of the c-myc gene with one of the immunoglobulin genes. The most common of these translocations is between chromosomes 8 and 14.

    In sub-Saharan Africa, Burkitt lymphoma of the jaw is associated with previous infection by the Epstein-Barr virus. This presentation of Burkitt lymphoma is called “endemic.” In other parts of the world, Burkitt lymphoma is not usually associated with Epstein-Barr virus.

    For the purpose of deciding how much treatment is necessary, Burkitt lymphoma may be divided into 3 groups:

    • Group A – Any localized tumor that has been completely removed by surgery
    • Group B – Any tumor that has not been removed by surgery and does not involve the nervous system or more than 25% of the bone marrow
    • Group C – Any lymphoma with at least 25% bone marrow involvement or with any brain or spinal involvement

    The level of an enzyme in the blood called LDH can be elevated in Burkitt lymphoma. Children with an LDH more than twice the normal level have higher risk disease.

    What are the treatments for Burkitt lymphoma?

    The lymphoma specialists at Dana-Farber/Boston Children’s determine the best approach to treatment for each child's unique situation, based on the type of lymphoma, the extent of the disease, the patient’s medical condition, the patient's and family’s preferences, and the most up-to-date medical knowledge about lymphoma therapies.

    For newly diagnosed Burkitt lymphoma or leukemia, treatment is based on the Group of the disease (as described in the classification section above).

    • Group A can be treated with a short course of chemotherapy lasting less than two months. Commonly used drugs are cyclophosphamide, vincristine, prednisone, and doxorubicin. 
    • Group B can be treated with about 4 months of chemotherapy. The same drugs that are used for treatment of Group A are used for Group B, with the addition of high-dose methotrexate and low-dose cytarabine. The immunotherapy drug rituximab (a monoclonal antibody that targets a marker called CD20 on the lymphoma cells) is often included in the treatment and has been shown to improve the outcome for children with a high LDH level in the blood. Multiple doses of chemotherapy into the spinal fluid are also an important part of the treatment.
    • Group C is treated with about 6 months of chemotherapy with the addition of high-dose cytarabine to the treatment plan. Rituximab is always included in the treatment of this group.

    If the correct treatment is selected, the cure rate for all three groups of Burkitt lymphoma is very high.

    Treatment of relapsed or refractory Burkitt lymphoma

    For Burkitt lymphoma that does not respond to initial treatment (refractory) or that comes back after treatment (relapse), there is no standard treatment recommendation. Refractory or relapsed Burkitt lymphoma is difficult to treat successfully.

    • One common treatment approach for relapsed or refractory Burkitt lymphoma is to give alternative chemotherapy (such as ifosfamide, carboplatin, and etoposide) and immunotherapy (Rituximab) followed by high dose chemotherapy and a stem cell transplant, either from the patient’s own bone marrow (autologous) or sometimes from another person’s bone marrow (allogeneic). The Dana-Farber/Boston Children’s Stem Cell Transplant Center offers autologous and allogeneic stem cell transplants and is active in researching new stem cell techniques.
    • An immunotherapy drug called Blinotumomab may be effective for treatment of this disease but has not been extensively tested.
    • Children who do not respond to established therapies may be eligible to receive experimental treatments on a clinical research trial.

    What is the latest Burkitt lymphoma research?

    For many children with cancer, clinical trials – research studies evaluating new treatment approaches – provide state-of-the art treatments and new opportunities for cure. Dana-Farber/Boston Children’s offers many clinical trials. It is possible your child will be eligible to participate in one of our lymphoma clinical trials. Participation in a clinical trial is optional and is never a requirement to receive treatment at Dana-Farber/Boston Children’s.

    In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG), the Therapeutic Advances in Childhood Leukemia and Lymphoma (TACL) group, and the Pediatric Oncology Experimental Therapeutics Investigators' Consortium (POETIC). We are the New England Phase I Center of the Children's Oncology Group.

    We have new treatments under investigation, both for newly diagnosed lymphomas and for lymphomas that have come back after initial treatment. New and experimental treatments include monoclonal antibody therapies, antibody-drug conjugates and bispecific antibodies that specifically target tumor cells; small molecule inhibitors that interfere with the way cancer cells grow; checkpoint inhibitors that activate the body’s own immune system to fight the cancer; and selective inhibitors of nuclear export that prevent tumor cells from escaping cell death. Clinical trials to investigate the biology of each child’s lymphoma are available and may help find targets of therapy unique to that lymphoma.

    Clinical Trials

    To learn more about the specific clinical trials that are currently available at the Dana-Farber/Boston Children's:

    What is the long-term outlook for children with Burkitt lymphoma?

    Despite its fast-growing nature, Burkitt lymphoma is one of the most curable forms of non-Hodgkin lymphoma. More than 90 percent of children with localized tumors and more than 85% with widespread disease are cured.

    Cancer survivorship care

    All kids who have been treated for cancer require ongoing, specialized care. Through the David B. Perini Jr. Quality of Life Clinic, our cancer survivorship clinic, childhood cancer survivors receive regular, comprehensive follow-up evaluations from their cancer care team. In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists, or alternative/complementary therapy specialists. We also offer patient and family education, psychosocial assessment, genetic counseling, reproductive counseling, and opportunities to speak with other childhood cancer survivors.

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