Treatment of Non-Hodgkin Lymphoma in Children, by Type and Stage
In general, all children with non-Hodgkin lymphoma are treated with chemotherapy, but the treatments differ depending on the type and stage of the lymphoma. This treatment is intense and might cause serious side effects, so it is very important that it is given in a children’s cancer center, especially when it is first started.
Lymphomas in children (especially Burkitt lymphomas) tend to grow very quickly and may already be quite large by the time they’re diagnosed, so it is important to start treatment as soon as possible. These lymphomas usually respond well to chemotherapy, which can kill large numbers of lymphoma cells in a short period of time. A concern is that this can cause tumor lysis syndrome, a side effect in which the inner contents of the dead cells enter the blood and can cause problems with the kidneys and other organs. Doctors try to prevent this by making sure the child gets lots of fluids before and during treatment, and by giving certain drugs to help the body get rid of these substances.
It is assumed even children with early stage (stage I or II) lymphomas have more widespread disease than can be detected with exams or imaging tests. Because of this, local treatments such as surgery or radiation therapy alone are very unlikely to cure them. Therefore, chemotherapy is an important part of treatment for all children.
Treatment of lymphoblastic lymphoma
Stages I and II: In general, treatment for these early stage lymphomas is similar to the treatment of acute lymphoblastic leukemia (ALL). Chemotherapy is given in 3 phases (induction, consolidation, and maintenance) using many drugs. For example, the BFM regimen uses combinations of many different drugs for the first several months, followed by less intense treatment with methotrexate and 6-mercaptopurine in pill form for a total of about 2 years. Shorter and less intensive treatments, such as the chemotherapy combinations called CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and COMP (cyclophosphamide, vincristine, methotrexate, and prednisone) have also been used.
Chemotherapy, usually with methotrexate, is also given into the spinal fluid (known as intrathecal chemo) for at least 4 doses, each separated by a week. This helps kill any lymphoma cells in the brain or spinal cord.
The total length of treatment may be as long as 2 years.
Stages III and IV: The treatment for children with advanced lymphoblastic lymphoma lasts for about 2 years. Treatment is typically more intensive than for earlier stage lymphomas. It is given as 3 phases of chemotherapy (induction, consolidation, and maintenance) using many drugs. This is very similar to the treatment of high-risk acute lymphoblastic leukemia (ALL). For more information, see Treating Childhood Leukemia.
Intrathecal chemotherapy is also given into the spinal fluid to kill any lymphoma cells that may have reached the brain or spinal cord. In some cases, radiation therapy may be given to the brain and spinal cord as well.
Treatment of Burkitt and Burkitt-like lymphoma
Chemotherapy is the main form of treatment for these lymphomas. Studies are now being done to determine whether adding a monoclonal antibody such as rituximab to chemotherapy will make treatment more effective.
Stages I and II: Treatment of these lymphomas may include surgery prior to chemotherapy if the tumor is in only one area. If there is a large abdominal tumor, it is important that as much of it as possible be removed. After that, chemotherapy is given.
Several different chemo drugs are used. The length of treatment ranges from about 9 weeks to 6 months. Most pediatric oncologists feel that the 9-week treatment is adequate if all of the tumor is removed with surgery first.
Chemotherapy into the spinal fluid is needed only if the lymphoma is growing around the head or neck.
Stages III and IV: Children with more advanced Burkitt lymphoma need more intensive chemotherapy. Because these lymphomas tend to grow quickly, the chemotherapy cycles are short, with little rest between courses of treatment.
For example, a treatment plan known as the French LMB protocol regimen alternates between different combinations of drugs every 3 to 4 weeks for a total of about 6 to 8 months. Other similar treatment regimens are the German BFM protocol and the St. Jude Total B regimen.
Chemotherapy must also be given into the spinal fluid.
Treatment of large cell (including anaplastic) lymphoma
Stages I and II: Treatment for these lymphomas usually consists of chemotherapy with 4 or more drugs given for around 3 to 6 months. For diffuse large B-cell lymphoma, treatment may include surgery in addition to chemotherapy. The usual chemotherapy regimen contains a 4-drug combination of cyclophosphamide, vincristine, prednisone, and either doxorubicin or methotrexate. (These are known as the CHOP or COMP regimens.)
Chemotherapy is given into the spinal fluid only if the lymphoma is near the head or neck.
Stages III and IV: Large cell lymphomas don’t often reach the bone marrow or spinal fluid, but if they do they require more intensive treatment.
Chemotherapy includes several drugs given over 9 to 12 months. Many doctors treat advanced large B-cell lymphomas as they would Burkitt lymphoma (see above).
Intrathecal chemotherapy is given into the spinal fluid as well.
Current clinical trials are focusing on the length of chemotherapy, which drugs are important in treating large cell lymphoma, and whether the different types of large cell lymphoma can be treated similarly. Newer drugs that might help treat anaplastic large cell lymphoma, such as brentuximab vedotin (Adcetris) and crizotinib (Xalkori), are also being studied.
Treatment of recurrent lymphoma
Generally, if the lymphoma comes back after the first therapy, it is much harder to treat. When possible, more intensive chemotherapy, usually including a stem cell transplant, is recommended. This is often done in a clinical trial. Clinical trials of newer forms of treatment may also be an option.
Last Medical Review: March 7, 2014 Last Revised: January 27, 2016
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- Radiation Therapy for Non-Hodgkin Lymphoma in Children
- Surgery for Non-Hodgkin Lymphoma in Children
- Treatment of Non-Hodgkin Lymphoma in Children, by Type and Stage
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