• Anaplastic Large Cell Lymphoma

    Anaplastic large cell lymphoma (ALCL) is a cancer of mature T-lymphocytes. It is a form of non-Hodgkin lymphoma.

    • About 10% of non-Hodgkin lymphoma cases in children are Anaplastic Large Cell Lymphoma.
    • Anaplastic large cell lymphoma can emerge in lymph tissue in the neck, chest, abdomen, lungs, skin, or bone.
    • When anaplastic large cell lymphoma is widespread, it can cause fevers, weight loss, and generalized symptoms of illness.

    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.

    Anaplastic Large Cell Lymphoma Treatment at Dana-Farber/Boston Children's

    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.

    How is anaplastic large cell lymphoma classified?

    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.

    What are the treatments for anaplastic large 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.

    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 prednisone.

    For advanced (stage III or IV) anaplastic large cell lymphoma, there are more than one standard treatment option. Two options that are equally effective are:

    • APO: a chemotherapy regimen that lasts for one year and can be given in an outpatient clinic. The treatment includes a higher total dose of a drug called doxorubicin and also includes several doses of chemotherapy drugs given into the spinal fluid by lumbar puncture. It does not include chemotherapy drugs called alkylators. The drugs in this regimen are doxorubicin, prednisone, vincristine, methotrexate and 6-mercaptopurine.
    • ALCL99: a chemotherapy regimen that lasts for 5 months. The treatment requires hospitalization for about one out of every 3 weeks. It includes a lower total dose of doxorubicin and only one dose of chemotherapy into the spinal fluid by lumbar puncture, but does include alkylators. The drugs in this regimen are doxorubicin, dexamethasone, cyclophosphamide, ifosfamide, methotrexate, cytarabine, and etoposide.

    Relapsed or 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.

    • One common treatment approach for relapsed or refractory anaplastic large cell lymphoma is to give alternative chemotherapy (such as ifosfamide, carboplatin and etoposide) followed by high dose chemotherapy and a stem cell transplant, either from the patient’s own bone marrow (autologous) or 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. There is some evidence to suggest that an allogeneic stem cell transplant may be more effective than an autologous stem cell transplant for anaplastic large cell lymphoma.
    • A chemotherapy drug called vinblastine has been shown to be an effective treatment for relapsed anaplastic large cell lymphoma.
    • The antibody-drug conjugate, brentuximab, has proven to be an effective treatment for some adults with relapsed or refractory anaplastic large cell lymphoma.
    • The ALK inhibitor drug crizotinib is another potential treatment, though it has not been around as long as some of the other treatment options.
    • Children who do not respond to established therapies may be eligible to receive experimental treatments on a clinical research trial.

    What is the latest research on anaplastic large cell lymphoma?

    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.

    Clinical Trials

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