Non-Hodgkin's Lymphoma

Non-Hodgkin's lymphoma is cancer of a type of white blood cell in the lymphatic system. This system includes the lymph nodes, spleen, and bone marrow. It drains fluid from tissue and returns it to the blood. It plays an important role in the body's defense against infection.

Non-Hodgkin's lymphoma is classified in several ways. One practical way to classify the disease is by how fast it grows. In this scheme, there are three grades: low-grade, intermediate-grade, and high-grade. Low-grade is the slowest-growing, and high-grade is the fastest.

What is going on in the body?

Non-Hodgkin's lymphoma begins in one type of lymph tissue. It starts either in the lymph nodes, the spleen, the bone marrow, or other less common lymph tissues. Lymph nodes are small, round glands found all over the body.

Lymphoma cells grow and cause lymph nodes and internal organs to enlarge. The lymphoma cells also infiltrate and crowd out the bone marrow. They can invade any other part of the body, including the brain, lungs, kidneys, and skin. They may also invade the hard part of the bone, the intestines, the liver, the spleen, or even the heart. Lymphomas can also cause immune system problems and unusual infections.

Autoimmune disorders are common in people with non-Hodgkin's lymphoma. Autoimmunity means that the body's immune system attacks itself. It may lead to kidney failure, anemia, blood clots, bleeding, arthritis, or nerve damage.

What are the causes and risks of the disease?

Lymphoma occurs when the genes of a white blood cell change enough that the cell grows uncontrollably. How this occurs is an important part of cancer research. Many of the genes that become abnormal and control this cell growth have been described. The exact process, as well as why some people develop lymphoma and others do not, remain unanswered parts of the cancer question.

Some of the risk factors for non-Hodgkin's lymphoma are:

Some autoimmune disorders may increase the risk of non-Hodgkin's lymphoma. Tobacco use does not seem to be a strong risk factor. There is controversy over whether some pesticides or herbicides can cause lymphoma. Non-Hodgkin's lymphoma occurs in all age groups and both genders. Some subtypes are more common in elderly people, and others are more common in young people.

What are the signs and symptoms of the disease?

Non-Hodgkin's lymphoma may grow slowly and cause few problems besides enlarged lymph nodes. The lymph nodes can be very large and may feel firm or rubbery. However, they are usually not tender to the touch. Other symptoms include:

A person with non-Hodgkin's lymphoma may develop anemia, or a low red blood cell count. The person may tire easily, have pale skin, and develop shortness of breath.

How is the disease diagnosed?

Diagnosis of non-Hodgkin's lymphoma begins with a medical history and physical exam. Lymphoma is diagnosed by biopsies, or the examining of tissue samples. Blood samples are taken so that immune studies can be done on white blood cells. Some lymphomas cause higher levels of white cells in the blood, but others do not.

Bone marrow biopsies and lymph node biopsies are usually used to make a diagnosis. Lymphomas are classified into different subtypes based on their characteristics. This classification of lymphomas continues to evolve as knowledge of the disease advances.

Lymphomas need to be staged, which is a rough measure of the amount of cancer in the body. Tests used in staging include:

Lymphoma stages are classified as I, II, III, and IV. This classification scheme is like the one for Hodgkin's lymphoma, another type of blood cancer.

Staging does not predict treatment outcomes like it does in Hodgkin's lymphoma. In non-Hodgkin's lymphoma, a better predictor is the type of tissue involved. Special laboratory testing called immune analysis can help predict the outcome of lymphoma. This analysis looks at cell proteins and gene abnormalities. It is not always helpful in figuring out which treatment to use, though.

What can be done to prevent the disease?

It's important to avoid radiation and toxins in the environment. These toxins include pesticides, insecticides, and solvents. There is no proof that the diet is important in preventing lymphoma. People whose medical problems put them at risk of developing lymphoma should see a healthcare provider regularly.

What are the long-term effects of the disease?

Long-term effects of non-Hodgkin's lymphoma depend on the histology, or cell type. Some people are completely cured, while others need periodic treatment when the cancer recurs. An individual with high-grade lymphoma may die quickly.

What are the risks to others?

Non-Hodgkin's lymphoma is not contagious and poses no risk to others.

What are the treatments for the disease?

Lymphoma tends to be a total-body or systemic disease. So chemotherapy is the mainstay of treatment. The exact medicines, doses, duration of treatment, and combination with radiation therapy depend on the type of lymphoma.

High-grade lymphomas are treated with intensive high-dose intravenous (IV) chemotherapy. This approach is sometimes used for intermediate-grade lymphomas, too. Methotrexate, vincristine, vinblastine, etoposide (VP-16), and many other medicines are used. They are given in cycles for several weeks to several months. People are usually treated as outpatients unless other medical problems arise.

Low-grade lymphomas are treated when problems occur. The problems usually result from long-term treatment with immune therapy or low-dose chemotherapy medicines, such as cladribine (2-cda) or fludarabine. It's not known for sure whether this disease can be cured with medical treatment. The problem has been that the disease recurs after treatment, and the recurrence is harder to treat. Lymphoma may need to be treated with high-dose chemotherapy, total-body or total-lymph node radiation, and bone marrow transplantation.

Surgery is sometimes needed, but it is not considered a cure. Surgery can relieve problems caused by lymphoma. These include bowel obstruction, blocked blood vessels, and spinal cord compression.

Radiation therapy is used to ease symptoms and as a potential cure. Early-stage lymphoma limited to one lymph node area can sometimes be cured with radiation alone. But radiation therapy alone does not work well on more advanced lymphomas. Radiation therapy is also used to shrink bothersome lymph nodes or other masses.

Immune therapy is giving a person manufactured proteins and other substances often made by a normal immune system. It's a new and evolving kind of treatment. Interferons, interleukins, monoclonal antibodies, immune toxins, or radiolabeled immune proteins may be given.

What are the side effects of the treatments?

Chemotherapy can cause:

Radiation therapy can cause:

These treatments also can cause damage to:

A combination of radiation and chemotherapy can increase the severity of side effects.

High-dose chemotherapy and bone marrow transplantation are highly toxic and risky treatments. Sometimes the transplanted immune system attacks the person's normal cells and tissue. There can be mild problems with skin rash and diarrhea. There can also be major organ failure, causing death.

What happens after treatment for the disease?

Some low-grade lymphomas are treated only to lessen the symptoms. Treatment is stopped when the benefits do not outweigh the side effects. Some people may need to be treated periodically. Other lymphomas are considered curable. Some high-grade lymphomas have a 60% to 80% cure rate.

How is the disease monitored?

Hodgkin's disease sometimes recurs. For this reason, the healthcare provider will monitor a person for several years by doing:

Any new or worsening symptoms should be reported to the healthcare provider.

Author:Thomas Fisher, MD
Date Written:
Editor:Crist, Gayle P., MS, BA
Edit Date:08/31/01
Reviewer:Eileen McLaughlin, RN, BSN
Date Reviewed:07/31/01