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Non-Hodgkin Lymphoma (NHL)

Overview
Symptoms
Risk Factors
Stages
Treatment Options
Types of NHL
Participating In Clinical Trials
Resources

 

Overview

Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Lymphoma occurs when lymphocytes, a type of white blood cell, grow abnormally. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood or other organs, and can accumulate to form tumors.

Non-Hodgkin lymphoma is the most common cancer of the lymphatic system, a part of the immune system. Since the early 1970’s, incidence rates for NHL have nearly doubled. Of the nearly 500,000 Americans with lymphoma, approximately 332,000 have this form. Over 65,000 cases of NHL are diagnosed annually in the United States.


Non-Hodgkin lymphoma is not a single disease, but rather a group of several closely related cancers. The World Health Organization estimates that there are at least 61 types of NHL. Although the various types of NHL have some things in common, they differ in their appearance under the microscope, their molecular features, their growth patterns, their impact on the body and how they are treated.
Non-Hodgkin lymphomas are broadly divided into two major groups: B-cell lymphomas and T-cell lymphomas. B-cell lymphomas develop from abnormal B-lymphocytes and account for 85 percent of all NHLs. T-cell lymphomas develop from abnormal T-lymphocytes and account for the remaining 15 percent of all NHLs. Non-Hodgkin lymphomas may also be classified as indolent (slow-growing) or aggressive (fast-growing).

Symptoms

Common signs and symptoms of NHL include swelling of the lymph nodes (which is often but not always painless), fever, night sweats, unexplained weight loss and lack of energy. While most people who have these complaints will not have NHL, anyone with persistent symptoms should be seen by a physician to make sure that lymphoma is not present.

Risk Factors

The causes of NHL remain unknown, however, risk for develop­ing lymphoma may be higher in individuals who:
•      have a family history of NHL (though no hereditary pattern has been well established)
•      are affected with an autoimmune disease
•      have received an organ transplant
•      have been exposed to chemicals such as pesticides, fertil­izers or organic solvents for a long period
•      have been infected with viruses such as Epstein-Barr, human T-lymphotropic virus type 1 (HTLV-1), HIV/AIDS, hepatitis C or certain bacteria, such as H-pylori

Stages

Non-Hodgkin lymphoma is divided into four stages based on how far the disease has spread.

      Stage I (early disease): the cancer is found only in a single lymph node OR in one organ or area outside the lymph node.
      Stage II (locally advanced disease): the cancer is found in two or more lymph node regions on one side of the diaphragm.
      Stage III (advanced disease): the cancer involves lymph nodes both above and below the diaphragm.
      Stage IV (widespread disease): the cancer is found in several parts of one or more organs or tissues (in addition to the lymph nodes). Or, it is in the liver, blood or bone marrow.

Treatment Options

Many effective treatment options exist for NHL patients, including:

•      watchful waiting
•      chemotherapy
•      radiation therapy
•      stem cell transplantation
•      novel targeted agents
•      newer versions of established agents

The form of treatment chosen depends on the type of lymphoma and the stage of disease, as well as other factors including age, prior therapies received and the patient’s overall health.

Some patients may relapse (disease returns after treatment) or become refractory (disease does not respond to treatment). However, numerous treatment options exist for patients with relapsed or refractory NHL, which are often referred to as secondary therapies. Many of the novel therapeutic agents that have been approved by the United States Food and Drug Administration, as well as those being investigated in clinical trials, focus specifically on those with relapsed or refractory disease.

Before starting treatment, patients should discuss all available treatment options with their physician.

Types of NHL

AIDS-Related Lymphomas
Anaplastic Large-Cell Lymphoma
Angioimmunoblastic Lymphoma
Blastic NK-Cell Lymphoma
Burkitt’s Lymphoma, Burkitt-like Lymphoma (Small Non-Cleaved Cell Lymphoma)
Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma
Cutaneous T-Cell Lymphoma
Diffuse Large B-Cell Lymphoma
Enteropathy-Type T-Cell Lymphoma
Follicular Lymphoma
Hepatosplenic Gamma-Delta T-Cell Lymphoma
Lymphoblastic Lymphoma
Mantle Cell Lymphoma
Marginal Zone Lymphoma
Nasal T-Cell Lymphoma
Pediatric Lymphoma
Peripheral T-Cell Lymphomas
Primary Central Nervous System Lymphoma
T-Cell Leukemias
Transformed Lymphomas
Treatment-Related T-Cell Lymphomas
Waldenstrom's Macroglobulinemia

AIDS-Related Lymphomas
Lymphomas occurring in HIV-positive patients are usually aggressive. It is estimated that as many as ten percent of people who are HIV-positive will ultimately develop lymphoma. Although both Hodgkin and non-Hodgkin lymphomas may occur in AIDS patients, non-Hodgkin lymphomas are more common and include diffuse large B-cell, Burkitt’s/Burkitt-like and primary central nervous system lymphoma.  More details and resources on Hodgkin lymphoma.

Anaplastic Large-Cell Lymphoma
Anaplastic large-cell lymphoma (ALCL) is a rare type of aggressive T-cell lymphoma comprising about 3 percent of all lymphomas in adults and between 10 percent and 30 percent of all lymphomas in children.  More details and resources.

 

Angioimmunoblastic Lymphoma
Angioimmunoblastic lymphoma (AILD) is a fast-growing T-cell lymphoma that accounts for between one percent and two percent of all cases of NHL in the United States. More details and resources.

 

Blastic NK-Cell Lymphoma
Blastic NK-cell lymphoma is a very rare T-cell lymphoma, affecting only a few people (usually adults) each year. This lymphoma is very fast growing, is difficult to treat and can arise anywhere in the body. Since this disease is so rare, patients should consult with their medical team to find promising therapies or clinical trials.  More details and resources on T-cell lymphoma.

 

Burkitt's Lymphoma, Burkitt-like Lymphoma (Small Non-Cleaved Cell Lymphoma)
Burkitt's lymphoma is an aggressive B-cell form of NHL that occurs most often in children and young adults. There are three main types of Burkitt’s lymphoma: sporadic, endemic and immunodeficiency-related disease. While sporadic Burkitt’s lymphoma occurs throughout most of the world, endemic Burkitt's lymphoma is found mostly in Africa and is often associated with the Epstein-Barr virus (EBV). Immunodeficiency-related Burkitt's lymphoma is diagnosed most often in people infected with HIV/AIDS. The disease may affect the jaw, central nervous system, bone marrow, bowel, kidneys, ovaries or other organs. Burkitt's lymphoma has a specific chromosomal abnormality called the t(8;14) translocation and behaves aggressively. Burkitt's lymphoma is potentially curable.  View Aggressive B-Cell Lymphoma Webcasts.

 

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma
According to the American Cancer Society, approximately 15,000 new cases of CLL and 3,600 new cases of SLL are diagnosed annually.  More details and resources.

 

Cutaneous T-Cell Lymphoma
Cutaneous T-cell lymphomas (CTCL) arise in the skin and account for approximately two percent to three percent of all NHL cases. More details and resources.

 

Diffuse Large B-Cell Lymphoma
Diffuse large B-cell lymphoma (DLBCL) is the most common form of NHL, accounting for up to one-third of newly diagnosed cases.  More details and resources.

 

Enteropathy-Type T-Cell Lymphoma
Enteropathy-type T-cell lymphoma is an extremely rare subtype of T-cell lymphoma that appears in the intestines and is strongly associated with celiac disease. As with other rare cancers, patients should discuss treatment options with their medical team.  More details and resources on T-cell lymphoma.

 

Follicular Lymphoma
Follicular lymphoma is a relatively common lymphoma, making up between 20 percent and 30 percent of all NHLs, and typically occurs in middle-aged and older adults, but it can affect younger people in their 30s and 40s.  More details and resources.

 

Hepatosplenic Gamma-Delta T-Cell Lymphoma
Hepatosplenic gamma-delta T-cell lymphoma is an extremely rare and aggressive disease that starts in the liver or spleen. This lymphoma may occur in people with Crohn’s disease whose immune system is suppressed. As with other rare cancers, patients should discuss treatment options with their medical team.  More details and resources on T-cell lymphoma.

 

Lymphoblastic Lymphoma
Lymphoblastic lymphoma can appear in both B-cells and T-cells, but is much more common in T-cells, comprising 80 percent of all lymphoblastic lymphomas. This lymphoma is most often diagnosed in children. With intensive chemotherapy, the complete remission rate can be very high. The disease is often treated similarly to acute lymphoblastic leukemia.  More details and resources on lymphoma in children and adolescents.

 

Mantle Cell Lymphoma
Mantle cell lymphoma is a B-cell lymphoma that affects approximately six percent of all NHL patients.  More details and resources

 

Marginal Zone Lymphoma
Marginal zone B-cell lymphomas, a group of indolent lymphomas whose cells come from B-lymphocytes normally found in the marginal zone of the secondary lymphoid follicles in the spleen and lymph nodes, accounts for approximately seven percent of all NHLs. The median age for diagnosis of this type of lymphoma is 65. Marginal zone lymphomas encompass three basic types: (1) extranodal or mucosa-associated lymphoid tissue (MALT), occurring outside the lymph nodes, (2) nodal, occurring within the lymph nodes, and (3) splenic, occurring mostly in the spleen and blood. Skin-associated lymphoid-tissue-related B-cell lymphoma (SALT) is also considered a form of MALT lymphoma. Marginal zone and MALT lymphomas vary from other types of B-cell NHLs in a number of ways: (1) their natural history is different; (2) many people who develop MALT lymphoma have a history of inflammation or autoimmune disorders; (3) chronic inflammation is associated with Helicobacter pylori (H. pylori), a microbial pathogen linked to chronic gastritis; and, (4) sometimes, MALT lymphomas can be treated with antibiotics. Different infections have also been implicated in other forms of MALT lymphoma. Hepatitis C has been associated with splenic marginal zone lymphoma. Nodal marginal zone B-cell lymphomas are uncommon and are sometimes called monocytoid B-cell lymphomas.

 


Nasal T-Cell Lymphoma
Although this fast-growing lymphoma is very rare in the United States, it is relatively common in Asia and parts of Latin America, leading researchers to suspect that some ethnic groups may be more prone to this cancer, which affects both children and adults. This type of lymphoma is associated with the Epstein-Barr virus. As with other rare cancers, patients should consult with their medical team for treatment options and the availability of clinical trials.  More details and resources on T-cell lymphoma.

 

Pediatric Lymphoma
Childhood NHL comprises about five percent of all NHL cases diagnosed in the United States. The most common types are lymphoblastic lymphoma, Burkitt’s lymphoma, diffuse large B-cell lymphoma and anaplastic large-cell lymphoma. Lymphoblastic lymphoma is closely related to childhood acute lymphoblastic leukemia. The number of children with NHL continues to increase.  More details and resources.

 

Peripheral T-Cell Lymphomas
Peripheral T-cell lymphomas (PTCL) refer to a large number of different T-cell lymphomas that together comprise between 10 percent and 15 percent of all NHL cases and can occur anytime during adulthood.  More details and resources.

 

Primary Central Nervous System Lymphoma
Primary central nervous system lymphoma (PCNSL) is a type of cancer that is limited to the brain or spinal cord but may also be found in tissues around the eye. An increasing occurrence of this disease has been seen in patients with AIDS and others whose immune system has been compromised. Median age of diagnosis for patients with PCNSL is 55 years for patients with a normal immune system and 31 years for AIDS patients. Although in the past the outlook for patients with this cancer has been poor, today, the survival rate has greatly improved.

 


T-Cell Leukemias
T-cell leukemias are also derived from T-cells and can act like T-cell lymphoma. These cancers include T-cell promyelocytic leukemia, T-cell granular lymphocytic leukemia, aggressive NK-cell leukemia and adult T-cell lymphoma/leukemia.

 


Transformed Lymphomas
Although indolent B-cell lymphomas, such as follicular lymphomas, are most commonly associated with transforming to aggressive disease, slow-growing T-cell lymphomas can also progress to aggressive disease.

 


Treatment-Related T-Cell Lymphomas
Treatment-related T-cell lymphomas may appear after solid organ or bone marrow transplantation. The immune system suppression that is required for transplant patients can put them at risk for developing post-transplant lymphoproliferative disorders, certain unusual forms of peripheral T-cell lymphoma and other types of NHL. Treatment-related T-cell lymphomas may require therapy that differs from the standard treatments normally used to treat these conditions.  More details and resources on T-cell lymphoma.

 

Waldenstrom's Macroglobulinemia
Waldenstrom’s macroglobulinemia (also known as lymphoplasmacytic lymphoma or immunocytoma) is a rare B-cell lymphoma that occurs in less than two percent of people with NHL.  More details and resources.

 

Participating in Clinical Trials

Clinical trials are crucial for identifying effective drugs for lymphoma patients. Patients inter­ested in participating in a clinical trial should talk to their physician. Contact the Lymphoma Research Foundation’s Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing helpline@lymphoma.org.

Resources

The Lymphoma Research Foundation has developed a number of resources, in a variety of formats, to provide relevant information to those diagnosed with NHL as well as their caregivers and loved ones.

Booklets/Fact Sheets
Webcasts/Podcasts
Teleconferences
In-person Educational Programs
Support Programs


For more information, call 1-800-500-9976, e-mail helpline@lymphoma.org, or continue to visit www.lymphoma.org.