Non-Hodgkin Lymphoma

Non-Hodgkin Lymphoma

Non-Hodgkin Lymphoma

Overview and Types

If you have been diagnosed with non-Hodgkin lymphoma or are worried about it, you likely have a lot of questions. Learning some basics is a good place to start. 

Types of B-cell Lymphoma

B-cell lymphomas account for the majority (approximately 85%) of non-Hodgkin lymphomas (NHL) in the United States. These types of lymphomas start in early forms of B lymphocytes (B cells). The most common types of B-cell lymphomas are listed below.

Diffuse large B-cell lymphoma (DLBCL)

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL) in the United States, accounting for approximately 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 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 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.

Follicular lymphoma

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. This lymphoma usually occurs in many lymph node sites in the body. It can also occur 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. Often, treatment may be delayed until the lymphoma starts causing problems. Over time, some follicular lymphomas can 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) lymphomas, although CLL, which is much more common, tends to grow more slowly. Treatment is the same for CLL and SLL. These lymphomas are usually not curable, but because they tend to grow slowly, they often don’t need to be treated when they are first found. Treatment can often be very helpful when it’s needed, and many people can live a long time (even decades) with CLL or SLL.

Sometimes, these lymphomas can turn into a more aggressive (fast-growing) type of lymphoma over time.

To learn more, see  Chronic Lymphocytic Leukemia.

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 hard 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. Newer treatments might offer a better chance for long-term survival for people being diagnosed with MCL.

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 sites).

There are gastric and non-gastric MALT lymphomas:

  • Gastric MALT lymphomas start in the stomach and are linked to infection with Helicobacter pylori (H. pylori), the bacteria that causes many stomach ulcers.
  • Non-gastric MALT lymphomas start outside the stomach, such as in the lungs, skin, thyroid, salivary glands, or tissues around the eye. The lymphoma usually stays in the area where it begins and doesn’t spread. Many of these 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 66. This lymphoma tends to grow slowly and is often curable if the cancer is not advanced.

Nodal marginal zone B-cell lymphoma: This is a rare type of lymphoma. It starts and usually stays in the lymph nodes, although lymphoma cells can sometimes be found in the bone marrow.

This lymphoma tends to be slow growing (although not usually as slow as MALT lymphoma), and it’s treated similarly to follicular lymphoma.

Splenic marginal zone B-cell lymphoma: This is a rare lymphoma. Often, the lymphoma is found mainly in the spleen, blood, and bone marrow.

It can cause tiredness 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 hepatitis C infection. Treatment for the hepatitis C virus is discussed in detail in Treating B-cell Non-Hodgkin Lymphoma.

Burkitt lymphoma

This fast-growing lymphoma is named after the doctor who first described this disease in African children and young adults. It makes up only about 1% to 2% of all adult lymphomas, but it’s 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, behaves much the same way, but the cells have different chromosome changes.

Different varieties of this lymphoma are seen in different parts of the world:

  • The variety seen most often in Africa (endemic Burkitt lymphoma) often starts as a tumor of the jaw or other facial bones. Most cases are linked to infection with the Epstein-Barr virus (EBV, which can also cause infectious mononucleosis or “mono”). This type of Burkitt lymphoma is rare in the United States.
  • In the type seen more often in the United States (nonendemic or sporadic Burkitt lymphoma), the lymphoma usually starts in the abdomen (belly), where it forms a large tumor. It can also start in the ovaries, testicles, or other organs, and can spread to the brain and spinal fluid. Some of these lymphomas are linked to EBV infection.
  • Immunodeficiency-associated Burkitt lymphoma is linked with immune system problems, such as in people with HIV or AIDS or who have had an organ transplant.

Burkitt lymphoma grows very quickly, so it needs to be treated right away. More than half of people with Burkitt lymphoma can be cured by intensive chemotherapy.

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 cancer cells are small B lymphocytes with projections coming off them that give them a “hairy” appearance. They are typically found in the bone marrow, 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). Sometimes the lymphoma is also found in tissues around the spinal cord. Over time, it tends to spread 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 might also have vision problems; weakness or altered sensation in the face, arms, or legs; and in some cases, seizures.

The outlook for people with primary CNS lymphoma has improved over the years mainly due to better treatments.

Primary intraocular lymphoma (lymphoma of the eye)

This is a rare type of lymphoma that starts in the eyeball and is often seen along with primary CNS lymphoma. Most people with primary intraocular lymphoma are elderly or have immune system problems that may be due to AIDS or anti-rejection drugs after an organ or tissue transplant.

People may notice bulging of the eyeball without pain, vision loss, or blurry vision. Many of the tests done to diagnose ocular melanoma are the same used to diagnose lymphoma of the eye.

The main treatment for lymphoma of the eye is external radiation therapy if the cancer is limited to the eye. Chemotherapy (chemo) or chemotherapy in combination with radiation may be used depending on the type of lymphoma and how far it has spread outside of the eye.

 

Types of T-cell Lymphoma

T-cell lymphomas make up less than 15% of non-Hodgkin lymphomas in the United States. These lymphomas start in early forms of T lymphocytes (T cells). There are many types of T-cell lymphoma, but they are all fairly rare.

T-lymphoblastic lymphoma/leukemia

Lymphoblastic lymphoma/leukemia (also known as T cell acute lymphoblastic leukemia/lymphoma) accounts for about 1% of all lymphomas. It’s most common in teens or young adults, with males being affected more often than females. It can be thought of as either a lymphoblastic lymphoma (LBL) or a type of acute lymphoblastic leukemia (ALL), depending on how much of the bone marrow is involved (leukemias have more cancer cells in the bone marrow). 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 fast-growing, but there is a good chance of curing it with chemotherapy if it hasn’t spread to the bone marrow when it is first diagnosed. Often, the lymphoma form of this disease is treated in the same way as the leukemia form.

Peripheral T-cell lymphomas

These are uncommon types of lymphoma that 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.

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:

  • The smoldering subtype tends to grow slowly. It often has no symptoms, and it tends to have a good prognosis (outlook).
  • The chronic subtype also grows slowly and tends to have a good prognosis.
  • The acute subtype is the most common. It grows quickly like acute leukemia, so it needs to be treated right away.
  • The lymphoma subtype grows more quickly than the chronic and smoldering types, but not as fast as the acute type.

Angioimmunoblastic T-cell lymphoma (AITL): This lymphoma accounts for about 4% of all lymphomas. It is more common in older adults. It tends to involve the lymph nodes and bone marrow, as well as the spleen or liver, which can become enlarged. People with this lymphoma usually have fever, weight loss, skin rashes, and often develop infections. This lymphoma often progresses quickly. Treatment often works 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. It is most common in the small intestine, but it can also occur in the colon. Symptoms can include severe abdominal (belly) pain, nausea, vomiting, and bleeding from the intestines.

This lymphoma occurs in some people with celiac disease (also called gluten-sensitive enteropathy). Celiac disease is an autoimmune disease in which eating gluten, a protein found mainly in wheat and barley, causes the immune system to attack the lining of the intestine and other parts of the body. This lymphoma is rare among people who have had celiac disease since childhood, but more common in people diagnosed as older adults. It is more common in men than women.

Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL): This rare type of lymphoma also affects the lining of the intestines, but it is not linked to celiac disease.

Anaplastic large cell lymphoma (ALCL): About 2% of lymphomas are of this type. It is more common in young people (including children), but it can also affect older adults. This type of lymphoma tends to be fast-growing, but many people with this lymphoma can be cured.

There are different forms of ALCL:

  • Primary cutaneous ALCL only affects the skin. This is discussed in more detail in Lymphoma of the Skin.
  • Systemic ALCL can affect the lymph nodes and other organs, including the skin. Systemic ALCL is divided into 2 types based on whether the lymphoma cells have a change in the ALK gene. ALK-positive ALCL is more common in younger people and tends to have a better prognosis (outlook) than the ALK-negative type.
  • Breast implant-associated ALCL is a rare type of ALCL that can develop in the breasts of women who have had implants. It seems to be more likely to happen if the implant surfaces are textured (as opposed to smooth).  

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS): This name is given to T-cell lymphomas that don’t fit well 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 also affect the skin, bone marrow, spleen, liver, and digestive tract. As a group, these lymphomas tend to spread and grow quickly. Some of these lymphomas respond well to chemotherapy, but over time, they tend to become harder to treat.

 

 

Key Statistics for Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma (NHL) is one of the most common cancers in the United States, accounting for about 4% of all cancers.

The American Cancer Society’s estimates for non-Hodgkin lymphoma in 2025 are:

  • About 80,350 people (45,140 males and 35,210 females) will be diagnosed with NHL. This includes both adults and children.
  • About 19,390 people will die from this cancer (11,060 males and 8,330 females).

Overall, the chance that a man will develop NHL in his lifetime is about 1 in 44; for a woman, the risk is about 1 in 54. But each person’s risk can be affected by a number of risk factors.

NHL can occur at any age. It is one of the more common cancers among children, teens, and young adults. Still, the risk of developing NHL increases throughout life, and more than half of people are 65 or older when they are first diagnosed.

Incidence rates have declined by about 1% per year for NHL since 2015. And from 2013 to 2022, the death rate decreased by about 2% per year.

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