What Is Childhood 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 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 progress quickly and children may 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.
Learn more about non-Hodgkin lymphoma, including causes and symptoms in children and teens.
How We Diagnose Childhood Burkitt Lymphoma
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 a marker called CD20. 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.
The level of an enzyme in the blood called LDH can be elevated in Burkitt lymphoma. Children with LDH levels that are more than twice the normal level have a higher risk of the disease.
For the purpose of deciding how much treatment is necessary, Burkitt lymphoma may be divided into three 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
How We Treat Childhood Burkitt Lymphoma
Children and teens with Burkitt lymphoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Childhood Lymphoma Program in our Childhood Hematologic Malignancy Center. Dana-Farber/Boston Children's offers internationally renowned care for children with cancers of the blood and immune system.
Dana-Farber/Boston Children's also offers a wide array of support services and programs for pediatric patients and their families during and after cancer treatment.
For newly diagnosed Burkitt lymphoma, treatment is based on the Group of the disease (as described in Diagnosis, 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 four 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 the CD20 marker on lymphoma cells) is often included in the treatment and has been shown to improve the outcome for children with high LDH blood levels. Multiple doses of chemotherapy into the spinal fluid are also an important part of the treatment.
- Group C is treated with about six 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 for Relapsed or Refractory Burkitt Lymphoma
There is no standard treatment recommendation for Burkitt lymphoma that does not respond to initial treatment (refractory) or that comes back after treatment (relapse). It is difficult to treat refractory or relapsed Burkitt lymphoma 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 your child'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 blinatumomab may be effective for treatment of Burkitt lymphoma 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.