Non-Hodgkin Lymphoma

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What Is Non-Hodgkin Lymphoma? TOPICS

Types of non-Hodgkin lymphoma

Classifying non-Hodgkin lymphoma can be quite confusing (even for doctors) because there are so many types and because several different systems have been used. The most recent system is the World Health Organization (WHO) classification. The WHO system groups lymphomas based on how they look under a microscope, the chromosome features of the lymphoma cells, and the presence of certain proteins on the surface of the cells. (Older systems classified lymphomas only by how they looked under a microscope.)

The more common types of lymphoma are listed below according to whether they are B-cell or T-cell lymphomas. Some rarer forms of non-Hodgkin lymphoma are not discussed here.

B-cell lymphomas

B-cell lymphomas make up most (about 85%) of non-Hodgkin lymphomas in the United States.

Diffuse large B-cell lymphoma

This is the most common type of non-Hodgkin lymphoma in the United States, accounting for about 1 out of every 3 cases. The cells are fairly large when seen using a microscope.

Diffuse large B-cell lymphoma (DLBCL) can affect any age group but occurs mostly in older people (the average age is mid-60s). It usually starts as a quickly growing mass in a lymph node deep inside the body, such as in the chest or abdomen, or in a lymph node that you can feel, such as in the neck or armpit. It can also start in other areas such as the intestines, bone, or even the brain or spinal cord.

About 1 in 3 of these lymphomas is confined to one part of the body (localized) when it is found. Lymphomas are easier to treat when they are localized than when they have spread to other parts of the body.

Genetic tests have shown that there are different subtypes of DLBCL, even though they look the same under the microscope. These subtypes seem to have different outlooks (prognoses) and responses to treatment.

DLBCL is a fast growing lymphoma, but it often responds well to treatment. Overall, about 3 out of 4 people will have no signs of disease after the initial treatment, and about half of all people with this lymphoma are cured with therapy.

Primary mediastinal B-cell lymphoma: This is a subtype of DLBCL in which the lymphoma cells are large but there is a lot of fibrosis (scar-like tissue) in the background. It accounts for about 2% of all lymphomas. About 2 out of 3 people with this lymphoma are women. Most are young – in their 30s.

This lymphoma starts in the mediastinum (the area in the middle of the chest behind the breastbone). It is usually localized when it is found. It can cause trouble breathing because it often presses on the windpipe (trachea) leading into the lungs. It can also block the superior vena cava (the large vein that returns blood to the heart from the arms and head), which can make the arms and face swell.

This is a fast-growing lymphoma, but it usually responds well to treatment. About half of patients can be cured.

Intravascular large B-cell lymphoma: In this rare subtype of DLBCL, the lymphoma cells are only found inside blood vessels, not in the lymph nodes or bone marrow. It is treated like DLBCL.

Follicular lymphoma

About 1 out of 5 lymphomas in the United States is follicular lymphoma. The term follicular means that the cells tend to grow in a circular pattern in lymph nodes.

The average age for people with this lymphoma is about 60. It’s rare in very young people. Most of the time, this lymphoma occurs in many lymph node sites in the body, as well as in the bone marrow.

Follicular lymphomas are often slow-growing and respond well to treatment, but they are hard to cure. These lymphomas may not require treatment when they are first diagnosed. Instead, treatment may be delayed until the lymphoma is causing problems. Over time, about 1 in 3 follicular lymphomas turns into a fast-growing diffuse B-cell lymphoma.

Chronic lymphocytic leukemia /small lymphocytic lymphoma

These are closely related diseases. In fact, many doctors consider them different versions of the same disease. The same type of cancer cell (known as a small lymphocyte) is seen in both chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL). The only difference is where the cancer cells are found. In CLL most of the cancer cells are in the blood and bone marrow. In SLL, the cancer cells are mainly in the lymph nodes and spleen. SLL accounts for about 5% to 10% of all lymphomas.

Both CLL and SLL are slow-growing diseases, although CLL, which is much more common, tends to grow more slowly. CLL and SLL are treated the same way. They are usually not curable with standard treatments, but depending on the stage and growth rate of the disease, most patients live longer than 10 years. Sometimes, these slow-growing lymphomas turn into a more aggressive type of lymphoma.

For more detailed information, see our document Leukemia -- Chronic Lymphocytic.

Mantle cell lymphoma

Only about 5% of lymphomas are this type. The cells are small to medium in size.

Men are affected most often. The average age of patients is in the early 60s. When this lymphoma is diagnosed, it is usually widespread in the lymph nodes, bone marrow, and often the spleen.

This usually isn’t a very fast-growing lymphoma, but it can be challenging to treat. Newer treatments might be more effective than those used in the past, and may offer a better chance for long-term survival for patients now being diagnosed.

Marginal zone B-cell lymphomas

Marginal zone lymphomas account for about 5% to 10% of lymphomas. The cells in these lymphomas look small under the microscope. There are 3 main types of marginal zone lymphomas.

Extranodal marginal zone B-cell lymphomas, also known as mucosa-associated lymphoid tissue (MALT) lymphomas: These lymphomas start in places other than the lymph nodes (extranodal) and are the most common type. Most MALT lymphomas start in the stomach and are linked to infection by the bacteria Helicobacter pylori, which is also the cause of stomach ulcers. Other possible sites of MALT lymphomas include the lung, skin, thyroid, salivary glands, and tissues surrounding the eye. Usually it is confined to the area where it begins and is not widespread. Many of these other MALT lymphomas have also been linked to infections with bacteria or viruses.

The average age of patients with MALT lymphoma is about 60. It is a slow-growing lymphoma and is often curable in its early stages. Doctors often use antibiotics as the first treatment for MALT lymphoma of the stomach, because treating the Helicobacter pylori infection often cures the lymphoma.

Nodal marginal zone B-cell lymphoma: This is a rare disease, found mainly in older women. It usually stays in the lymph nodes, although lymphoma cells can also sometimes be found in the bone marrow.

This tends to be a slow-growing lymphoma (although not usually as slow as MALT lymphoma), and many patients are cured if they are diagnosed when the disease is in the early stages.

Splenic marginal zone B-cell lymphoma: This is a rare lymphoma. Most often the lymphoma is found only in the spleen and bone marrow.

Patients are often elderly and male and have fatigue and discomfort caused by an enlarged spleen. Because the disease is slow-growing, treatment may not be needed unless the symptoms become troublesome. This type of lymphoma has been linked to infection with the hepatitis C virus.

Burkitt lymphoma

This type makes up about 1% to 2% of all lymphomas. It is named after the doctor who first described this disease in African children and young adults. The cells are medium sized. Another kind of lymphoma, called Burkitt-like lymphoma, has slightly larger cells. Because this second kind of lymphoma is hard to tell apart from Burkitt lymphoma, the WHO classification combines them.

This is a very fast-growing lymphoma. In the African (or endemic) variety, it often starts as tumors of the jaws or other facial bones. This type is linked to infection with the Epstein-Barr virus (which can also cause infectious mononucleosis or “mono”). The endemic type of Burkitt lymphoma is rare in the United States. In the types seen more often in the United States, the lymphoma usually starts in the abdomen, where it forms a large tumor mass. It can also start in the ovaries, testicles, or other organs, and can spread to the brain and spinal fluid. The type seen in the United States is usually not linked to Epstein-Barr viral infection.

Close to 90% of patients are male, and the average age in the US is about 30. Although this is a fast-growing lymphoma, more than half of patients can be cured by intensive chemotherapy.

Lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia)

This type is not common, accounting for 1% to 2% of lymphomas. The lymphoma cells are small and found mainly in the bone marrow, lymph nodes, and spleen. It is discussed in detail in our document Waldenstrom Macroglobulinemia.

Hairy cell leukemia

Despite the name, this is sometimes considered to be a type of lymphoma. Hairy cell leukemia (HCL) is rare – about 700 people in the United States are diagnosed with it each year. The cells are small B lymphocytes with projections coming off them that give them a “hairy” appearance. They are typically found in the bone marrow and spleen and in the blood.

Men are more likely to get HCL than women, and the average age is around 50.

Hairy cell leukemia is slow-growing, and some patients may never need treatment. An enlarging spleen or dropping blood cell counts (due to cancer cells invading the bone marrow) are the usual reasons to begin treatment. If treatment is needed, it’s usually very effective.

Hairy cell leukemia is also described in our document Leukemia--Chronic Lymphocytic

Primary central nervous system (CNS) lymphoma

This lymphoma usually involves the brain (called primary brain lymphoma), but it may also be found in the spinal cord and in tissues around the spinal cord and the eye. Over time, it tends to become widespread in the central nervous system.

Primary CNS lymphoma is rare overall, but it’s more common in people with immune system problems, such as those infected with HIV, the virus that causes AIDS. Most people develop headaches and confusion. They can also have vision problems; weakness or altered sensation in the face, arms, or legs; and in some cases, seizures.

The outlook for people with this condition has always been thought to be fairly poor, but some people can live at least 5 years with today’s treatments.

Lymphoma of the eye (primary intraocular lymphoma), which is related to primary CNS lymphoma, is discussed in our document Eye Cancer (Melanoma and Lymphoma).

T-cell lymphomas

T-cell lymphomas make up less than 15% of non-Hodgkin lymphomas in the United States. There are many types of T-cell lymphoma, but they are all fairly rare.

Precursor T-lymphoblastic lymphoma/leukemia

This disease accounts for about 1% of all lymphomas. It can be considered either a lymphoma or leukemia, depending on how much of the bone marrow is involved (leukemias have more bone marrow involvement). The cancer cells are small-to-medium sized, immature T-cells.

This lymphoma often starts in the thymus (see the image below). This is where many T cells are made. This lymphoma can develop into a large tumor in the mediastinum (the area in the middle of the chest and behind the breast bone). If the tumor presses on the windpipe (trachea) that carries air into the lungs, it can cause trouble breathing. The tumor can also press on or even block the superior vena cava (the large vein that returns blood to the heart from the arms and head), which can make the arms and face swell.

Patients are most often young adults, with males being affected more often than females.

This lymphoma is fast-growing, but if it hasn’t spread to the bone marrow when it is first diagnosed, the chance of cure with chemotherapy is quite good.

Often, the lymphoma form of this disease is treated in the same way as the leukemia form. For more information, see our document Leukemia - Acute Lymphocytic (Adults).

Peripheral T-cell lymphomas

These types of lymphomas develop from more mature forms of T cells. They are rare, accounting for only a small portion of all lymphomas.

Cutaneous T-cell lymphomas (mycosis fungoides, Sezary syndrome, and others): These lymphomas start in the skin. Skin lymphomas account for about 5% of all lymphomas. They are described in our document Lymphoma of the Skin.

Angioimmunoblastic T-cell lymphoma: This lymphoma accounts for about 3% of all lymphomas. It tends to occur in the lymph nodes and may affect the spleen or liver. Patients usually have fever, weight loss, and skin rashes and often develop infections. This lymphoma often progresses quickly. Treatment is often effective at first, but the lymphoma tends to come back.

Extranodal natural killer/T-cell lymphoma, nasal type: This rare type often involves the upper airway passages, such as the nose and upper throat, but it can also invade the skin and digestive tract. It is much more common in parts of Asia and South America. Cells of this lymphoma are similar in some ways to normal natural killer (NK) cells. NK cells are a kind of lymphocyte that can respond to infections more quickly than T-cells and B-cells.

Enteropathy type intestinal T-cell lymphoma: This lymphoma occurs in people with extreme sensitivity to gluten, the main protein in wheat flour. People with this disease, called gluten-sensitive enteropathy (celiac sprue or sprue), can develop lymphoma in the walls of the intestines. This is rare in people found to have sprue in childhood, and is more often found in adults either recently diagnosed with sprue or who didn’t realize that they had sprue until part of the intestine was removed to treat the lymphoma. Many experts believe that these patients had sprue for a long time without knowing it. It is thought that if sprue is found early, following a gluten-free diet may help prevent the lymphoma from developing. People who do not tolerate gluten, but don’t have sprue, do not seem to have an increased risk of this type of lymphoma.

Anaplastic large cell lymphoma (ALCL): About 2% of lymphomas are of this type. It is more common in young people (including children), but it does occur in people in their 50s and 60s. It usually starts in lymph nodes and can also spread to skin. This type of lymphoma tends to be fast-growing, but many people with this lymphoma are cured with aggressive chemotherapy. There are 2 main forms of ALCL, systemic ALCL and primary cutaneous ALCL, which only affects the skin. Systemic ALCL is divided into 2 types based on whether a gene change is present in the lymphoma cells that causes them to make a lot of protein called anaplastic lymphoma kinase or ALK1. ALK-positive ALCL tends to have a better prognosis (outlook) than the ALK-negative type.

Primary cutaneous ALCL isn’t discussed further is this document, but is described in our document Lymphoma of the Skin.

Peripheral T-cell lymphoma, unspecified: This name is given to T-cell lymphomas that don’t readily fit into any of the groups above. They make up about half of all T-cell lymphomas. The tumor cells can be small or large. Most people diagnosed with this disease are in their 60s. As a group, these lymphomas tend to be widespread and grow quickly. Some cases respond well to chemotherapy, but long-term survival is not common.

Last Medical Review: 03/27/2013
Last Revised: 07/30/2014