• Highlights
• Introduction
• Risk Factors
• Symptoms
• Diagnosis
• Outlook
• Staging and Treatment Guidelines
• Chemotherapy
• Biologic Therapy (Immunotherapy)
• Radiation
• Transplantation
• Surgery
• Resources
• References

Non-Hodgkin's lymphoma

Description

An in-depth report on the causes, diagnosis, and treatment of non-Hodgkin's lymphoma.

Alternative Names

Lymphoma - non-Hodgkin's; NHL; B-cell lymphomas

Outlook

Five-year survival rates for NHL range from 20 - 95% depending on the lymphoma type, stage, age of the patient, and other variables. Because the outlook varies so widely, making a definite prognosis is very difficult. For example, patients with very slow growing (indolent) lymphomas can live many years. However, they are usually diagnosed at a late stage, when the cancer has spread, which reduces the survival rate. Aggressive lymphomas are more likely to cause rapid death, but they are also often curable. New drugs that target specific factors in the tumor cells are improving survival rates.

Outlook for Indolent (Low-Grade) Lymphomas

Follicular lymphomas, the most common indolent (slow-growing) NHLs, are potentially curable in early stages I and II. Unfortunately, however, these slow-growing malignancies produce no symptoms until they are in advanced stages. In most cases, these lymphomas are not diagnosed until they have spread to other sites, including the spleen and bone marrow. In such cases, they are difficult to cure. Predicting outcome for indolent follicular lymphomas is more difficult than for aggressive lymphomas. Even if treatment achieves a response, these tumors almost always recur. Even after relapse, however, the tumors can be treated again if they are still very slow-growing.

In general, the average survival rate for follicular lymphoma is 7 - 10 years, depending on other risk factors. New drug treatments, particularly monoclonal antibodies, have significantly improved survival rates. According to a 2005 study, 91% of patients with follicular lymphoma now survive the first 4 years after diagnosis, compared with 69% of patients treated in the past with older types of drugs. The research team found the best 4-year survival rates for patients treated with the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy regimen followed by monoclonal antibody biologic drugs (rituximab or iodine-131 tositumomab).

Factors for Predicting Outlook in Indolent Lymphomas. Six risk factors are proving to be useful for predicting outlook:

• Being male
• Being older
• Having stage III or IV disease
• Elevated levels of the enzyme lactate dehydrogenase (LDH)
• The presence of B symptoms
• Erythrocyte sedimentation rate over 30

Patients with a good chance for a positive outcome (65% chance for survival rates of 10 years or greater) have one or none of these factors; those with intermediate risk (23%) have two factors, and those likely to have a poor outcome (11%) have three or more factors. MALT lymphomas generally have a good prognosis. Primary gastric lymphomas have a 3-year survival rate of 89%.

Outlook for Aggressive (Intermediate- to High-Grade) Lymphomas

High-grade aggressive lymphomas are often symptomatic early on and are potentially curable with aggressive treatments. Diffuse large-cell lymphomas, the most common aggressive non-Hodgkin's lymphomas, while fatal if not treated, are often curable with intensive chemotherapy combinations. If relapse occurs after chemotherapy, it usually does so within 2 years.

Most other aggressive lymphomas respond to aggressive chemotherapy. Mantle cell lymphoma is less responsive to chemotherapy. The average survival time is 3 - 5 years.

Factors for Predicting Outlook in Aggressive Lymphomas: A scoring system called the International Prognostic Index has proved to be fairly accurate for predicting outcome in patients with most aggressive B-cell lymphomas. It uses five risk factors to help predict whether the disease will be aggressive:

• Being older than 60 -- this age group tends to have other medical conditions, which contribute to the poorer prognosis
• Having a disseminated tumor (stage III or IV)
• Disease that has spread to more than one site beyond the lymph nodes
• A poor performance status
• Having elevated levels of lactate dehydrogenase (LDH)

Having one or none of these risk factors indicates the best outlook; two factors indicate a low to intermediate likelihood of a poor outlook; three factors predict an intermediate to high likelihood of poor outlooks; and four or five factors pose the highest likelihood of poor survival.

Risk Factors for Central Nervous System Involvement

Lymphoma can spread to the central nervous system or it can appear there first, which is referred to as primary CNS lymphomas (PCNSL). It is a very serious event, particularly if it occurs at relapse.

Risk Factors for CNS Involvement After a Diagnosis of NHL. According to a 2002 study, the risk for CNS involvement in low-grade NHL is about 2.8%. Among patients with high-grade NHL, those with lymphoblastic and Burkitt's lymphoma are at high risk (nearly 25%) for recurrence in the central nervous system. For other patients with high-grade NHL, the 5-year risk was slightly over 5%, although having other factors increased this danger.

Risk Factors of Primary CNS Lymphomas. PCNSL used to account for only about 2% of lymphomas, but the incidence is on the rise in all age groups and in both. The reason for the increase is not known.

Long-Term Complications of Treatments

Medical Problems. The radiation and chemotherapies used in treating NHL can have long-term effects on many organs in the body and can increase the risk for serious illnesses, including heart disease and certain cancers.

Negative Emotional Problems. Depression and anxiety are common in survivors, particularly those who suffer additional medical conditions. Many patients also suffer from fatigue and aches and pains, called somatic symptoms, which have no apparent physical basis. In one study, such symptoms were more highly associated with intensive chemotherapy. Women and people in lower social and economic groups are at higher risk for depression and somatic symptoms -- just as they are in the general population.

• Review Date: 1/17/2007
• Reviewed By: Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital.

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