Primary mediastinal B-cell lymphoma (PMBL) 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
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.
What are the treatments for primary mediastinal B-cell 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 and family’s preferences, and the most up-to-date
medical knowledge about lymphoma therapies.
Primary mediastinal B-cell lymphoma has a protein, called
CD20, on the surface of the cancer cells. Rituximab is an immunotherapy drug that
targets the CD20 protein. Modern treatments for PMBL include rituximab, along
with chemotherapy. Traditionally, radiation therapy has been part of treatment
for this disease, but with the addition of rituximab, radiation may no longer
be a necessary part of the treatment.
The most commonly used treatment regimen for primary
mediastinal B-cell lymphoma, both in adolescents and in adults, is called
dose-adjusted EPOCH-R. This chemotherapy regimen is given as a 96-hour long
continuous infusion into an intravenous line, once every three weeks for six
courses. The doses of the chemotherapy drugs are adjusted according to blood
tests that are done in between treatment courses. The drugs that are included
in this treatment are etoposide, prednisone, vincristine, cyclophosphamide,
doxorubicin, and rituximab. A drug called filgrastim is used to help the body’s
white blood cells recover quickly after each course of chemotherapy.
refractory primary mediastinal B-cell lymphoma
For primary mediastinal B-cell lymphoma that does not respond
to initial treatment (refractory) or that comes back after treatment (relapse),
treatment recommendations are the same as for other relapsed or refractory mature
B-cell lymphomas, such as Burkitt lymphoma.
If radiation therapy was not part of the initial treatment,
it may be recommended to treat refractory primary mediastinal B-cell lymphoma.
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