Non-Hodgkin lymphoma is cancer in the lymphatic system. According to estimates by the Leukemia and Lymphoma Society, in 2010, children younger than 15-years-old were expected to make up 4.3% of all newly diagnosed cases of non-Hodgkin lymphomas.
The lymphatic system is part of the immune system, and functions to fight disease and infections.
The lymphatic system includes:
Non-Hodgkin lymphoma causes the cells in the lymphatic system to abnormally reproduce, eventually causing tumors to grow. Non-Hodgkin disease cells can also spread to other organs and tissues in the body.
Lymphomas are the fifth most common childhood cancer. They occur most often in children between 7-years-old and 11-years-old but can occur at any age from infancy to adulthood.
It affects males almost three times more often than females and is of the disease and the specific cells involved.
Non-Hodgkin lymphoma in children is almost always one of three subtypes:
Lymphoblastic non-Hodgkin lymphoma accounts for about 35% of the cases, involves the T-cells, and usually presents with a mass in the chest, swollen lymph node(s), with or without bone marrow and central nervous system involvement.
Burkitt or non-Burkitt lymphoma is a non-Hodgkin disease in which the B-cells are undifferentiated and diffuse. These have also been referred to as small non-cleaved cells. Burkitt and non-Burkitt lymphoma accounts for about 40 to 50 percent of the cases and is usually characterized by a large abdominal tumor and may have bone marrow and central nervous system involvement.
Large cell or diffuse histiocytic non-Hodgkin can involve both the B-cells and T-cells and accounts for about 15 to 20 percent of the cases. Children with this type of non-Hodgkin lymphoma usually have lymphatic system involvement, as well as a non-lymph structure (i.e., lung, jaw, brain, skin, and bone) involvement.
Large cell anaplastic lymphoma is a less common type of lymphoma in children. Treatment for this type is the same as for large cell lymphoma.
The specific cause is unclear. It is possible that genetics and exposure to viral infections may increase the risk for developing this malignancy. Non-Hodgkin lymphoma has also been linked to chemotherapy and radiation therapy. Non-Hodgkin may be a second malignancy as a result of the treatment for certain cancers.
There has been much investigation into the association of the Epstein-Barr virus (EBV) that causes the mononucleosis infection; as well as the human immunodeficiency virus (HIV), which causes acquired immune deficiency syndrome (AIDS). Both of these infectious viruses have been linked to the development of Burkitt lymphoma.
The majority of Burkitt lymphoma cases result from a chromosome rearrangement between chromosome nonhodgkins1.asp#8 and nonhodgkins1.asp#14, which causes genes to change positions, and function differently, promoting uncontrolled cell growth. Other chromosome rearrangements have been seen in non-Hodgkin lymphoma (all types) that are also thought to promote excessive cell growth.
Children and adults with other hereditary abnormalities have an increased risk of developing non-Hodgkin lymphoma, including patients with ataxia telangiectasia, X-linked lymphoproliferative disease or the Wiskott-Aldrich syndrome.
Most children have stage III or IV disease at the time of diagnosis. The disease can progress quickly from a few days to a few weeks. A child can go from otherwise healthy to having multi-system involvement in a short time period. Some children with non-Hodgkin lymphoma have symptoms of an abdominal mass and have complaints of abdominal pain, fever, constipation and decreased appetite — due to the pressure and obstruction a large tumor in this area can cause.
Some children with non-Hodgkin lymphoma have symptoms of a mass in their chest and have complaints of respiratory problems, pain with deep breaths (dyspnea), cough and/or wheezing.
Because of the rapid onset of this malignancy, any respiratory symptoms can quickly worsen, causing a life-threatening emergency.
The following are the most common symptoms of non-Hodgkin lymphoma. However, each child may experience different symptoms.
Symptoms may include:
The symptoms of non-Hodgkin lymphoma may resemble other blood disorders or medical problems.
In addition to a complete medical history and physical examination, diagnostic procedures may include:
Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging symptoms that are used for non-Hodgkin lymphoma. Always consult your child's physician for information on staging. One method of staging non-Hodgkin lymphoma is the following:
Specific treatment will be determined by your child's physician based on:
Treatment may include (alone or in combination):
Prognosis greatly depends on:
As with any cancer, prognosis and long-term survival can vary greatly from child to child. Every child is unique and treatment and prognosis are structured around the child. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for the child diagnosed with non-Hodgkin's lymphoma. Side effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of non-Hodgkin's lymphoma. New methods are continually being discovered to improve treatment and to decrease side effects.