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.
Lymphomas in children (especially Burkitt lymphomas) tend to grow very quickly and may be quite large when diagnosed, so it is important to start treatment as soon as possible. These lymphomas usually respond well to chemotherapy, which can lead to the death of large numbers of lymphoma cells in a short period of time. 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.
Treatment of lymphoblastic lymphoma
Stages I and II: All children with stages I and II lymphoblastic lymphoma are assumed to have more widespread disease than might be visible with imaging tests. Because of this, localized treatments such as surgery or radiation therapy aren't likely to cure these lymphomas.
Treatment is with systemic chemotherapy using combinations of several drugs. For example, doctors may use combinations such as CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) or COMP (cyclophosphamide, vincristine, methotrexate, and prednisone) for a few months, followed by less intense treatment with methotrexate and 6-mercaptopurine in pill form for several months. Many doctors now feel that combinations containing even more drugs, similar to the treatment of acute lymphoblastic leukemias, might be more successful. For example, a combination called the BFM regimen uses 8 drugs for the first 2 months followed by less intense treatment with methotrexate and 6-mercaptopurine in pill form for a total of about 2 years.
Intrathecal chemotherapy, usually with methotrexate, is also given into the spinal fluid for at least 4 doses, each separated by a week. This chemotherapy helps kill any lymphoma cells in the brain or spinal cord.
The total length of treatment may be as long as 2 years. Around 85% to 90% of these children will be cured.
Stages III and IV: The treatment for children with advanced lymphoblastic lymphoma is lengthy, lasting 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 multiple drugs. This is very similar to the treatment of high-risk acute lymphoblastic leukemia (for more information, see the treatment section of the document, 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.
The cure rate for these lymphomas is generally higher than 80%.
Treatment of non-cleaved cell (Burkitt and non-Burkitt) 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 in addition to chemotherapy. If there is a large abdominal tumor, it is important that as much as possible be removed. After that, chemotherapy is given.
The length of treatment ranges from about 9 weeks to 6 months. Usually several drugs are used, most commonly prednisone, vincristine, cyclophosphamide, ara-C, doxorubicin, and methotrexate. Most pediatric oncologists feel that the 9-week treatment is adequate. Chemotherapy into the spinal fluid is needed only if the lymphoma is growing around the head or neck.
Treatment of these lymphomas is usually very successful, with a cure rate of over 90%.
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 prednisone, cyclophosphamide, doxorubicin, and vincristine with cytarabine (ara-C) and methotrexate every 3 to 4 weeks for a total of 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.
The cure rate for children with this type and stage of non-Hodgkin lymphoma ranges from about 80% to 90%.
Treatment of large cell (including anaplastic) lymphoma
Chemotherapy is the main form of treatment for these lymphomas. Studies are being done to determine whether adding rituximab or other monoclonal antibodies to chemotherapy might make treatment more effective.
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 cyclophosphamide, vincristine, prednisone and doxorubicin or methotrexate. Chemotherapy is given into the spinal fluid only if the lymphoma is near the head or neck.
The cure rate is over 90% for diffuse large B-cell lymphomas and slightly lower for anaplastic large cell lymphomas.
Stages III and IV: Large cell lymphomas don't often involve the bone marrow or spinal fluid, but if they do they require more intensive treatment.
Chemotherapy is usually doxorubicin, prednisone, vincristine, methotrexate, and possibly 6-mercaptopurine or cyclophosphamide over 9 to 12 months. Some doctors treat advanced large B-cell lymphomas as they would small non-cleaved cell lymphoma (see above). Intrathecal chemotherapy is given into the spinal fluid as well.
Current clinical trials are focusing on the length of treatment, which drugs are important in treating large cell lymphoma, and whether the different types of large cell lymphoma can be treated similarly.
The cure rate for children with advanced diffuse large B-cell lymphoma ranges from about 80% to 90%. For advanced anaplastic large cell lymphoma, the cure rate is about 60% to 75%.
Treatment of recurrent lymphoma
Generally, if the lymphoma comes back after curative 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 the context of a clinical trial. Clinical trials of newer forms of treatment may also be an option.
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