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Non-Hodgkin Lymphoma

What is non-Hodgkin lymphoma (NHL)?

Non-Hodgkin lymphoma is cancer in the lymphatic system. According to estimates by the Leukemia and Lymphoma Society, 88 percent of lymphomas diagnosed in the US in 2007 were non-Hodgkin lymphoma. The lymphatic system is part of the immune system and functions to fight disease and infections.

The lymphatic system includes the following:

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 the ages of 7 and 11, but can occur at any age from infancy to adulthood.

Non-Hodgkin lymphoma affects males more often than females, and is more common among Caucasian children than among African-American children and children of other races.

Staging and classification of non-Hodgkin lymphoma is based on the extent of the disease and the specific cells involved.

What are the different types of non-Hodgkin lymphoma?

Non-Hodgkin lymphoma in children is almost always one of three types:

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.

How is non-Hodgkin lymphoma staged?

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:

What causes non-Hodgkin lymphoma?

The specific cause of non-Hodgkin lymphoma 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's lymphoma.

The majority of Burkitt's lymphoma cases result from a chromosome rearrangement between chromosome #8 and #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 Wiskott-Aldrich syndrome.

What are the symptoms of non-Hodgkin lymphoma?

Most children have stage III or IV disease at the time of diagnosis because of the sudden onset of symptoms. 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 the symptoms differently. Symptoms may include:

The symptoms of non-Hodgkin lymphoma may resemble other blood disorders or medical problems. Always consult your child's physician for a diagnosis.

How is non-Hodgkin lymphoma diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for non-Hodgkin lymphoma may include:

Treatment of non-Hodgkin lymphoma:

Specific treatment for non-Hodgkin lymphoma will be determined by your child's physician based on:

Treatment may include (alone or in combination):

Long-term outlook for a child with non-Hodgkin lymphoma:

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 is 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 lymphoma. Side effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of non-Hodgkin lymphoma. New methods are continually being discovered to improve treatment and to decrease side effects.

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