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.
Children with non-Hodgkin lymphoma are treated at
Dana-Farber/Boston Children's through the Lymphoma
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.
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.
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 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.
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:
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.