Non-Hodgkin Lymphoma

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What Is Non-Hodgkin Lymphoma? TOPICS

Types of non-Hodgkin lymphoma

Classifying non-Hodgkin lymphoma (NHL) 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 the cells 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, (about 1 out of every 3 cases). The cells look fairly large when seen with 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 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 only in 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 outcomes (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 many 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.

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. About 5% to 10% of all lymphomas are SLLs.

Both CLL and SLL are slow-growing diseases, although CLL, which is much more common, tends to grow more slowly. Treatment is the same for CLL and SLL. 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.

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

Mantle cell lymphoma

Only about 5% of lymphomas are this type. The cell size is small to medium.

Men are affected most often. The average age of patients is 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 Helicobacter pylori, which is the bacteria that causes many 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 lymphoma tends to be slow-growing (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, they might not need treatment 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, 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 a tumor of the jaw or other facial bones. It 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 only 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.

Adult T-cell leukemia/lymphoma: This lymphoma is caused by infection with a virus called HTLV-1. It is rare in the United States, and much more common in Japan, the Caribbean, and parts of Africa – where the HTLV-1 virus is more common. There are 4 subtypes: smoldering, chronic, acute, and lymphoma.

The smoldering subtype has abnormal T-cells in the blood without an increased number of lymphocytes in the blood. This lymphoma may involve the skin or lungs, but there is no involvement of other tissues. The smoldering type grows slowly and has a good prognosis.

The chronic subtype also grows slowly and has a good prognosis. It has an increase in total lymphocytes and T-cells in the blood. It may involve the skin, lungs, lymph nodes, liver, and/or spleen, but nor other tissues.

The acute subtype acts like acute leukemia. It has high lymphocyte and T-cell counts, often along with enlargement of lymph nodes, liver, and spleen. The skin and other organs may be involved with lymphoma as well. Patients often have fever, night sweats, and/or weight loss, as well as certain abnormal blood test results.

The lymphoma subtype grows more quickly than the chronic and smoldering types, but not as fast as the acute type. It has enlarged lymph nodes without increased lymphocytes in the blood, and the T-cell count is not high.

Angioimmunoblastic T-cell lymphoma: This lymphoma accounts for about 3% of all lymphomas. It is more common in older adults. It tends to involve the lymph nodes as well as the spleen or liver, which can cause them to be enlarged. 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 (recur).

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 lymphocytes that can respond to infections more quickly than T-cells and B-cells.

Enteropathy-associated intestinal T-cell lymphoma (EATL): EATL is a lymphoma that occurs in the lining of the intestine. This lymphoma is most common in the jejunum (the second part of the small intestine), but can also occur elsewhere in the small intestine and in the colon. EATL often affects more than one place in the intestine, and may spread to the nearby lymph nodes, as well. It can cause the intestine to become blocked (obstruction) or a hole to develop in the intestine (a perforation). If either of these occur, the symptoms can include severe abdominal (belly) pain, nausea, and vomiting. There are 2 subtypes of this lymphoma.

  • Type I EATL occurs in people with a disease called gluten-sensitive enteropathy (also known as celiac disease, celiac sprue, or sprue). Sprue is an autoimmune disease in which gluten, the main protein in wheat flour, causes the body produce antibodies that attack the lining of the intestine and other parts of the body. Type I EATL is rare among people who have had sprue since childhood, and is more often seen in people diagnosed as adults who have had sprue for a long time. It is most common in people with sprue that hasn’t been well controlled on a gluten free diet. Still, type I EATL can sometimes occur in people who didn’t realize that they had sprue before the lymphoma was found. This lymphoma is more common in men than women, and tends to occur in people in their 60s and 70s. People who do not tolerate gluten, but don’t have sprue, do not seem to have an increased risk of this type of lymphoma.
  • Type II EATL is not linked to sprue and looks different than type I under the microscope. Type II EATL is less common than type I.

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.

The main forms of ALCL are primary cutaneous, which only affects the skin, and systemic. 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 occur in younger patients and 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. This lymphoma often involves lymph nodes, but it can affect the skin, bone marrow, liver, and GI (gastrointestinal) tract, as well. As a group, these lymphomas tend to be widespread and grow quickly. Some patients respond well to chemotherapy, but long-term survival is not common.


Last Medical Review: 08/26/2014
Last Revised: 09/15/2014