Types of Non-Hodgkin Lymphoma
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There are many different types of non-Hodgkin lymphoma (NHL), so classifying it can be quite confusing (even for doctors). Several different systems have been used, but the most recent system is the World Health Organization (WHO) classification. The WHO system groups lymphomas based on:
B-cell lymphomas make up most (about 85%) of NHL in the United States.
Diffuse large B-cell lymphoma (DLBCL)
This is the most common type of NHL in the United States, accounting for about 1 out of every 3 lymphomas. The lymphoma cells look fairly large when seen with a microscope.
DLBCL can affect people of any age, but it occurs mostly in older people. (The average age at the time of diagnosis 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, bones, or even the brain or spinal cord.
DLBCL tends to be a fast-growing (aggressive) 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.
A common subtype of DLBCL is primary mediastinal B-cell lymphoma. This type of lymphoma occurs mostly in young women. It starts in the mediastinum (the area in the middle of the chest behind the breastbone). It can grow quite large and 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.
There are several other subtypes of DLBCL, but these are rare.
About 1 out of 5 lymphomas in the United States is a follicular lymphoma. This is usually a slow-growing (indolent) lymphoma, although some follicular lymphomas can grow quickly.
The average age for people with this lymphoma is about 60. It’s rare in very young people. Usually, this lymphoma occurs in many lymph node sites throughout the body, as well as in the bone marrow.
Follicular lymphomas often respond well to treatment, but they are hard to cure. These lymphomas may not need to be treated when they are first diagnosed. Instead, treatment may be delayed until the lymphoma starts causing problems. Over time, some follicular lymphomas turn into a fast-growing diffuse large B-cell lymphoma.
Chronic lymphocytic leukemia (CLL) /small lymphocytic lymphoma (SLL)
CLL and SLL 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 CLL and 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.
Both CLL and SLL are usually slow-growing (indolent) 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 many people can live a long time (even decades) with them. Sometimes, these can turn into a more aggressive (fast-growing) type of lymphoma over time.
Mantle cell lymphoma (MCL)
About 5% of lymphomas are mantle cell lymphomas. MCL is much more common in men than in women, and it most often appears in people older than 60. When MCL is diagnosed, it is usually widespread in the lymph nodes, bone marrow, and often the spleen.
MCL can be challenging to treat. It tends to grow faster than indolent (slow-growing) lymphomas, but it doesn’t usually respond to treatment as well as aggressive (fast-growing) lymphomas. But newer treatments might offer a better chance for long-term survival for patients now being diagnosed.
Marginal zone lymphomas
Marginal zone lymphomas account for about 5% to 10% of lymphomas. They tend to be slow-growing (indolent). The cells in these lymphomas look small under the microscope. There are 3 main types of marginal zone lymphomas:
Extranodal marginal zone B-cell lymphoma, also known as mucosa-associated lymphoid tissue (MALT) lymphoma: This is the most common type of marginal zone lymphoma. It starts in places other than the lymph nodes (extranodal). Most MALT lymphomas start in the stomach and are linked to infection by Helicobacter pylori (the bacteria that causes many stomach ulcers). MALT lymphoma might also start in the lung, skin, thyroid, salivary glands, or tissues surrounding the eye. Usually the lymphoma stays in 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 people with MALT lymphoma at the time of diagnosis is about 60. This lymphoma tends to grow slowly and is often curable if found early. 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 starts and 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 is often curable if found early.
Splenic marginal zone B-cell lymphoma: This is a rare lymphoma. Most often the lymphoma is found only in the spleen and bone marrow.
It is most common in older men, and often causes fatigue and discomfort due to an enlarged spleen. Because the disease is slow-growing, it might not need to be treated unless the symptoms become troublesome. This type of lymphoma has been linked to infection with the hepatitis C virus.
This fast-growing lymphoma is named after the doctor who first described this disease in African children and young adults. It makes up about 1% to 2% of all lymphomas. It is rare in adults, but is more common in children. It’s also much more common in males than in females.
The cells in Burkitt lymphoma are medium-sized. A similar kind of lymphoma, Burkitt-like lymphoma, has slightly larger cells. Because it is hard to tell these lymphomas apart, the WHO classification combines them.
Different varieties of this lymphoma are seen in different parts of the world:
Lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia)
This slow-growing lymphoma 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. This lymphoma is discussed in detail in Waldenstrom Macroglobulinemia.
Hairy cell leukemia
Despite the name, hairy cell leukemia (HCL) is sometimes considered to be a type of lymphoma. It is rare – about 700 people in the United States are diagnosed with it each year. Men are much more likely to get HCL than women, and the average age at diagnosis is around 50.
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.
Hairy cell leukemia is slow-growing, and some people may never need treatment. An enlarging spleen or low 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 talked about in Chronic Lymphocytic Leukemia.
Primary central nervous system (CNS) lymphoma
This lymphoma involves the brain or spinal cord (the central nervous system, or CNS). The lymphoma is also sometimes found in tissues around the spinal cord or 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 older people and in people with immune system problems, such as those who have had an organ transplant or who have 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.
Historically, the outlook for patients with primary CNS lymphoma has not been as good as for many other lymphomas, but this is at least partly because people with CNS lymphoma tend to be older or have other serious health problems. Still, some people do well with treatment.
For information about lymphoma of the eye (primary intraocular lymphoma), which is related to primary CNS lymphoma, see Eye Cancer (Melanoma and Lymphoma).
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 a type of acute lymphoblastic leukemia (ALL), depending on how much of the bone marrow is involved (leukemias have more bone marrow involvement). The cancer cells are very early forms of T cells.
This lymphoma often starts in the thymus (a small organ behind the breastbone and in front of the heart, which is where many T cells are made), and can grow into a large tumor in the mediastinum (the area between the lungs). This can cause trouble breathing and swelling in the arms and face.
This lymphoma is most common in teens or 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 Acute Lymphocytic Leukemia (Adults).
Peripheral T-cell lymphomas
These uncommon types of lymphomas develop from more mature forms of T cells.
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 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 infection with HTLV-1 is more common. It can affect the bone marrow (where new blood cells are made), lymph nodes, spleen, liver, skin, and other organs. There are 4 subtypes:
Angioimmunoblastic T-cell lymphoma: This lymphoma accounts for about 4% 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 become enlarged. People with this lymphoma 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, digestive tract, and other organs. It is much more common in parts of Asia and South America. Cells of this lymphoma are similar in some ways to natural killer (NK) cells, another type of lymphocyte.
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 small intestine, but can also occur in the colon. Symptoms can include severe abdominal (belly) pain, nausea, and vomiting. There are 2 subtypes of this lymphoma:
There are different forms of ALCL:
Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS): This name is given to T-cell lymphomas that don’t readily fit into any of the groups above. Most people diagnosed with these lymphomas are in their 60s. These lymphomas often involve the lymph nodes, but they can affect the skin, bone marrow, spleen, liver, and digestive tract, as well. As a group, these lymphomas tend to be widespread and grow quickly. Some patients respond well to chemotherapy, but over time these lymphomas tend to become harder to treat.