Description: Lymphoma is a form of cancer of the lymph glands. There are 2 types of lymphoma: Hodgkin's lymphoma and non-Hodgkin's lymphoma. Lymphomas usually show up as enlarged lymph glands. In contrast to the movable and sometimes painful lymph glands associated with infection, the lymphomatous lymph glands are usually non-movable and are most often non-tender. The most commonly involved lymph glands in lymphoma are found in the neck and/or the chest, but any lymph gland, including those inside of the abdomen, can be involved. The best way to diagnose lymphoma is to do a biopsy of the swollen lymph gland. That is to surgically remove a piece of the gland for examination under the microscope. Sometimes, chromosome analysis and cell marker studies are also needed to determine the lymphoma type. Other tests that may help the doctor in finding out just how big a tumor burden that the patient has (stages I-IV) include: complete blood counts, blood chemistries, chest X-ray, gallium scan, CTor MRI scans of the chest and abdomen/pelvis, bone marrow biopsy and spinal tap.

Stage I - localized to one region of the body only

Stage II - involving 2 separate regions but contained on the same side of the diaphragm (the partition between the chest and the abdomen)

Stage III - disease on both sides of the diaphragm

Stage IV- disease involving liver, bone marrow and/or central nervous system The doctor needs to know the extent of the disease (stage) in order to plan an appropriate treatment program. Children with Hodgkin's lymphoma who exhibit the following symptoms (B symptoms) usually have more advanced disease and a worse prognosis: temperature 101degree F, drenching night sweats, or 10 lb weight loss over 6 months. Children with non-Hodgkin's lymphoma who have either bone marrow or central nervous system involvement would also, in general, have a worse prognosis.

Treatment for Hodgkin's Lymphoma: Even though radiation therapy is usually the treatment of choice for the adult patients with Hodgkin's disease, chemotherapy is usually chosen over radiation therapy for the treatment of children, because children with fast growing bones and soft tissues are more sensitive to effects of radiation therapy. If radiation therapy is used (as in the case of a large tumor in the chest that is difficult to sterilize by chemotherapy alone, or the presence of B symptoms , or having stages III/IV disease), the dose of radiation given would be reduced almost by half to prevent stunted growth or deformed bones from developing at the site of radiation treatment. The medicine (chemotherapy) used in the treatment of Hodgkin's disease include: alternating courses of vincristine, prednisone, nitrogen mustard, procarbazine (MOPP) and adriamycin, bleomycin, vinblastine and dacarbazine (ABVD) for 6 months (stages I/II) to 12 months (stages 2B, III/IV).

Treatment for non-Hodgkin's lymphoma (NHL): Children with NHL tend to have advanced disease with a higher incidence of either bone marrow and/or central nervous system involvement. The treatment of choice is, therefore, more ntensive chemotherapy. Because there are 2 types of NHL, the treatments for these 2 types are also quite different. The 2 types of NHL are called either B-cell NHL or T-cell NHL, depending on the type of lymphocyte (a type of white blood cell) that has turned cancerous. Treatment for B-cell NHL includes Adriamycin, Ara-C, cyclophosphamide, ifosfamide, methotrexate, vincristine and vinblastine given as an injection into the vein in various combinations and time intervals. Ara-C and methotrexate are also given as an injection into the cerebrospinal fluid (the liquid surrounding the brain and the spinal cord) through a spinal tap to prevent the disease from spreading there and as treatment for the disease that has already spread there. Treatment for T-cell NHL includes Adriamycin, cyclophosphamide, L-asparaginase, methotrexate, and vincristine given as an injection into the vein, with mercaptopurine and prednisone given by mouth in various combinations and time intervals. Ara-C and methotrexate are also given as an injection into the cerebrospinal fluid (the liquid surrounding the brain and the spinal cord) through a spinal tap to \prevent the disease from spreading there and as treatment for the disease that has already spread there. Radiation therapy to the head for the prevention and treatment of disease involving the central nervous system.

Because of the excellent response to chemotherapy, bone marrow transplantation is being used experimentally for patients who are resistant to chemotherapy or whose lymphoma recurred after initial response to chemotherapy.

There are 2 types of bone marrow transplantation: allogeneic bone marrow transplantation (AlloBMT) is when a marrow donor is used and autologous bone marrow transplantation (ABMT), when the patients' own bone marrow is given back to the patient.

1. Not everybody has a compatible marrow donor. There is only a 25% chance of finding a compatible marrow donor among one's siblings - the best source for marrow donors. The chances drop to 5% among other blood relatives and 1:1,000,000 among unrelated donors.

2. Depending on the experience of the transplantation team, there is 5-20% death rate associated with the bone marrow transplantation, either from infection, graft rejection (the patient rejects the donor's cells) or acute graft versus host disease (the donor's cells attack the patient's bodily organs/systems).

3. Late complications may include: rigid lung, damaged heart muscles, infertility, chronic graft versus host disease and recurrence of the lymphoma.

The problem with ABMT is the recurrence of lymphoma after bone marrow transplantation.

Prognosis:Depending on the stage of the disease, children with Hodgkin's disease have a 70-90% chance long term survival, B-cell NHL, 80% chance, and T-cell NHL, 70% . As with all cancers, treatment decisions need to be individualized based on many factors. Especially with pediatric malignancies, it is very important that patients seek the care of Pediatric Oncologists who are familiar with state-of-the-art therapies. Many cancers which were uniformally fatal ten years ago are curable today. The revolution in molecular biology is accelerating the pace of progress. Many exciting new therapies are being investigated. Some of these treatments will likely become the standards for treatment in the near future.