Anaplastic large cell lymphoma (ALCL) is a cancer of mature
T-lymphocytes. It is a form of non-Hodgkin
All anaplastic large cell lymphoma
tumors express a protein marker called “CD30”. Most, but not all, have a
rearrangement of a gene called “ALK”. These can be tested for in the laboratory.
Although uncommon, children with ALK-negative anaplastic large cell lymphoma have the same treatment and
outcome as those with ALK-positive lymphoma.
Children with anaplastic large cell 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.
For the purpose of deciding how much treatment is necessary,
anaplastic large cell lymphoma may be grouped by the extent, or stage, of the
disease. When the lymphoma is only in one area of the body (stage 1 or 2) it is
called localized. When it is more extensive (stage 3 or 4) it is called
advanced. With current therapies, more than 70% of children with Anaplastic
Large Cell Lymphoma are cured of the 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.
Newly diagnosed, localized (stage I or II) anaplastic large
cell lymphoma may be treated with a short 9-week course of chemotherapy,
including the drugs cyclophosphamide, doxorubicin, vincristine and
For advanced (stage III or IV) anaplastic large cell lymphoma,
there are more than one standard treatment option. Two options that are equally
refractory anaplastic large cell lymphoma treatment
For anaplastic large cell lymphoma that does not respond to
initial treatment (refractory) or that comes back after treatment (relapse),
there is no standard treatment recommendation. However, about half of children
with relapsed ALCL can be cured with second line therapy.
There is a new antibody-drug combination called Brentuximab
that targets the CD30 protein on the anaplastic large cell lymphoma cancer
cells and brings a cytotoxic chemotherapy drug directly to the cancer cells.
This drug has been shown to be effective in the treatment of ALCL that does not
respond to initial chemotherapy or that comes back after treatment. Brentuximab
is being studied in combination with the ALCL99 chemotherapy regimen for
children with newly diagnosed stage II, III or IV ALCL.
A drug called crizotinib interferes with the abnormal ALK
protein in ALCL cells that overexpress ALK. Crizotinib has been shown to be
effective in children with chemotherapy resistant or recurrent ALK-positive
ALCL. This drug also is being tested in combination with the ALCL99 chemotherapy
regimen for children with newly diagnosed stage II, III or IV ALCL.
Both of these newer drugs hold the promise to improve
treatment outcomes for children with anaplastic large cell lymphoma.
To learn more about the specific lymphoma clinical trials
that are currently available at the Dana-Farber/Boston Children’s:
Phone: 617-632-5508Online form: Request an appointment
World class cancer care at the #1 Children's Hospital