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About Childhood 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. Anaplastic large cell lymphoma cases are divided into two types: ALK-negative anaplastic large cell lymphoma and ALK-positive anaplastic large cell lymphoma. Children with ALK-negative anaplastic large cell lymphoma have the same treatment and outcome as those with ALK-positive lymphoma.

    Learn more about non-Hodgkin lymphoma, including causes and symptoms in children and teens.

    How Anaplastic Large Cell Lymphoma is 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.

    Learn how non-Hodgkin lymphoma is diagnosed and staged.

    How We Treat Childhood Anaplastic Large Cell Lymphoma

    Children and teens with anaplastic large cell lymphoma, a type of non-Hodgkin 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.

    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 is more than one standard treatment option. Two options that are equally effective are:

    • APO: This is 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: This is 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.

    Treatment for Relapsed or Refractory Anaplastic Large Cell Lymphoma

    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.

    Research and Clinical Trials

    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.

    For many children with cancer, clinical trials (research studies evaluating new treatment approaches) provide state-of-the-art treatments and new opportunities for cure. We offer many lymphoma clinical trials, and your child may be eligible to participate in one. Participation in a clinical trial is optional and is never a requirement to receive treatment.

    Learn more about clinical trials for pediatric cancer.

    Long-term Outlook for Children with Anaplastic Large Cell Lymphoma

    Childhood cancer was once considered to be invariably fatal, but today, the majority of children diagnosed with cancer can expect to be long-term survivors.

    Along with survivorship come numerous complex issues: the long-term effects of treatment and the risk of second cancers, as well as social and psychological concerns. For these reasons, survivors of childhood anaplastic large cell lymphoma should receive regular follow-up monitoring and care.

    Since 1993, physicians, nurses, researchers, and psychologists in our pediatric cancer survivorship programs at the David B. Perini Jr. Quality of Life Clinic at Dana-Farber have helped thousands of childhood cancer survivors, treated at Dana-Farber/Boston Children's and at other hospitals in New England and elsewhere, to manage these long-term challenges of surviving cancer.

    Childhood Anaplastic Large Cell Lymphoma Treatment Team

    Dana-Farber/Boston-Children's patients have access to the broadest set of pediatric hematologic and oncologic expertise available. The breadth of our expertise allows us to assemble a team of specialists to meet the specific needs of your child.

    See a complete list of the specialists in our Childhood Hematologic Malignancy Center.