Facts

This disease affects NK cells and causes skin lesions. It is rare in the United States and more common in Asia and parts of Latin America. It usually strikes older adults and is linked with Epstein-Barr virus. The main treatment is chemotherapy. Because this subtype of NHL is severe and hard to treat, patients should get care at an academic center that can transition them to a bone marrow transplant.

There are three types of this rare, aggressive T-cell lymphoma.

• Primary cutaneous ALCL, one of several types of cutaneous lymphoma.
• ALK-positive (ALK+) ALCL, body-wide disease which expresses a protein called anaplastic lymphoma kinase (ALK). It usually affects children.
• ALK-negative (ALK-) ALCL, body-wide disease which doesn’t express ALK. It is typically found in adults over age 60.

Both ALK+ and ALK- are treated as aggressive lymphomas. Patients with ALK+ ALCL tend to have excellent outcomes with the standard chemotherapy known as CHOP (cyclophosphamide, doxorubicin hydrochloride, vincristine and prednisone). In 2011, the drug brentuximab vedotin (Adcetris) was approved for patients with both ALK+ and ALK- relapsed or refractory systemic ALCL.

AITL is an aggressive subtype. It is typically diagnosed at stage III or IV. People with AITL usually have fever, night sweats, weight loss, skin rashes and autoimmune disorders, and they often develop infections. Physicians don’t know what causes AITL, but it has been linked with Epstein-Barr virus. To start, treatment usually involves steroids. It often progresses and may be treated with the typical T-cell NHL treatments — chemotherapy or other medicine-based options  — as well as radiation therapy.

This is an aggressive subtype of B-cell NHL found mostly in young men. The average age is 30. In the United States, the disease usually starts as a large tumor in the belly. It can also start in the testicles, ovaries or other organs. Patients are usually treated with chemotherapy. More than half enter complete remission.

CLL and SLL are closely related and are a subtype of B-cell NHL. 

• In CLL, the cancer cells are mainly in the blood and bone marrow. 
• In SLL, the cancer cells are mainly in the lymph nodes and spleen. 

Along with common NHL symptoms, CLL/SLL may cause shortness of breath, anemia, bruising and frequent infections.

Both are slow growing. You and your physician may decide to monitor the disease rather than treat it. This is called watchful waiting.If you do need active treatment, it could include chemotherapy and antibody therapy. If the disease does not respond to therapy or it becomes fast growing form, your physician may recommend a bone marrow transplant.

This is also known as lymphoma of the skin and is a subtype of . It mainly affects lymphocytes in the skin but may involve lymph nodes, blood and other organs.

It is nearly always slow growing. The chance of recovery is usually very good. Many people have a relapse after responding to treatment at first.

Treatment may be:

• Directed at the skin. This includes surgery, radiation therapy, phototherapy (using ultraviolet light) and medicines put on the skin.

Directed at the whole body (systemic treatments). This includes photopheresis, interferon, targeted therapies, chemotherapy or other medicines and bone marrow transplant.

DLBCL is the most common subtype of NHL. It is also a subtype of B-cell NHL. DLBCL makes up about 30 percent of NHLs in the United States. 

There are several subtypes:

• Primary mediastinal B-cell lymphoma. This is typically only in the space in the chest between the lungs.
• Intravascular large B-cell lymphoma. This is typically throughout the body.
• Primary effusion lymphoma. This is a rare subtype linked with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS).

DLBCL advances quickly. Treatment usually starts right away with chemotherapy and the antibody therapy rituximab (Rituxan). After their initial treatment, most people enter long-term remission. In some people, the disease doesn’t respond to treatment (refractory disease), or it gets better but then gets worse (relapsed disease). Then high-dose chemotherapy and a bone marrow transplant may be an option.

This extremely rare subtype affects the intestines. It happens mainly in middle-aged men who are sensitive to gluten, the main protein in wheat flour. It develops when celiac sprue (gluten-sensitive enteropathy) progresses to cancer. There is no standard treatment. Treatments can include surgery to remove the affected part of the intestine, chemotherapy and bone marrow transplant. A gluten-free diet might prevent this lymphoma.

This subtype is more common in parts of Asia and South America. It is linked with Epstein-Barr virus, which causes mononucleosis (often called mono). It often involves the upper airways, such as the nose and upper throat. It can also invade the skin and gastrointestinal tract. This is the only peripheral T-cell lymphoma subtype for which radiation therapy is the main treatment.

Follicular means the cells tend to grow in a circle. This subtype of B-cell NHL is often slow growing. It makes up about 20 percent of NHLs and affects many lymph nodes throughout the body as well as the bone marrow. About 30 percent of the time, it transforms into aggressive diffuse B-cell lymphoma.

Since it typically grows slowly, you and your physician may decide to monitor the disease rather than treat it. This is known as watchful waiting.

If you do need active treatment, it could include chemotherapy, radiation therapy or immunotherapy. Many patients enter long-term remission after treatment. But in most people the disease gets better but then gets worse (relapsed disease) or doesn’t respond to treatment (refractory disease). Then options include high-dose chemotherapy and a bone marrow transplant or treatments being tested in clinical trials.

This is called leukemia but is sometimes thought of as lymphoma.It is a subtype of B-cell NHL and gets its name from the way the cells look. They have fine structures on their surface that make them look hairy. The disease is rare, with fewer than 1,000 people diagnosed in the United States each year.

It is slow growing. Treatment may not be needed in the early stages. Some patients need a blood transfusion from time to time. If your spleen becomes enlarged or your blood counts drop, your options may include surgery to remove the spleen and chemotherapy.

This extremely rare, aggressive disease starts in the liver or spleen. It can cause fever, abdominal pain, weakness and swelling of the liver and spleen. It happens mainly in children, young men and people whose immune system is suppressed (usually due to an organ transplant). There is no standard treatment. One option is chemotherapy using the medicine anthracycline. This causes a short-term response in most patients. Complete remission is rare. Physicians typically recommend a bone marrow transplant using a donor’s stem cells (allogeneic transplant) the first time a patient goes into remission.

In this subtype of B-cell NHL, abnormal lymphocytes build up, forming nodules around the body, most often the lungs. The nodules damage or destroy blood vessels in the area. This subtype is linked to infection with Epstein-Barr virus, which causes mononucleosis (often called mono).

If the disease has low impact on your health, you and your physician may decide to monitor the disease rather than treat it. This is called watchful waiting. If you do need active treatment, options include interferon, corticosteroids, chemotherapy and the antibody therapy rituximab (Rituxan).

MCL typically affects men between the ages of 50 and 70. It has often spread throughout the body by the time it is diagnosed. It may be in the lymph nodes, bone marrow, blood, digestive tract and spleen.

This B-cell NHL subtype can be hard to label as either slow growing or fast growing. Physicians often approach MCL as fast growing (aggressive) because it is hard to treat.

Treatment may include chemotherapy combined with immunotherapy. Bone marrow transplant is an option for some people. Many treatments are being studied in clinical trials.

Marginal zone lymphomas are slow growing. They are typically diagnosed in people in their 60s. There are three main subtypes.

Extranodal marginal zone B-cell lymphoma (mucosa-associated lymphoid tissue lymphoma, MALT)

This starts outside the lymph nodes. Usually it is only in the area where it began. Gastric MALT develops in the stomach. It may be linked with an infection. Antibiotics for the infection are typically the first treatment. They often put the cancer into complete remission. If the cancer relapses, physicians may use immunotherapy, chemotherapy, radiation therapy or surgery. Non-gastric MALT develops in the lungs, skin, thyroid, salivary glands and tissues near the eye. Treatment depends on where the disease is. It usually involves surgery or radiation therapy.

Nodal marginal zone B-cell lymphoma

This disease, found mainly in women, usually stays in the lymph nodes. Lymphoma cells may also be in the bone marrow. Treatment often begins with watchful waiting. If you need active treatment, options include radiation therapy and chemotherapy.

Splenic marginal zone B-cell lymphoma

This subtype of NHL, found in the bone marrow, blood and spleen, is rare. It is typically found in men and has been linked with hepatitis C. Treatment may include watchful waiting unless symptoms become a problem. If you need active treatment, options include surgery to remove the spleen, radiation therapy to the spleen, immunotherapy and chemotherapy.

Physicians use this name for T-cell lymphomas that don’t fit easily into any of the other subtypes of peripheral T-cell lymphoma. These lymphomas tend to be widespread and aggressive. Most people diagnosed are in their 60s. Most are treated with chemotherapy.

This rare B-cell NHL subtype is more common in people with immune system diseases, such as HIV/AIDS. It often involves the brain (primary brain lymphoma) and may be found in and around the spinal cord and eyes (primary intraocular lymphoma).

Over time, it spreads throughout the central nervous system (brain and spinal cord). The symptoms are different than for most lymphomas. Symptoms may include headaches, confusion, vision problems, facial paralysis and seizures.

To start, people with this subtype may receive corticosteroids to control symptoms and reduce swelling. Other treatments include chemotherapy, radiation therapy and occasionally bone marrow transplant.

People with this rare, aggressive disease usually have small bumps or lesions under their skin. It’s most common in people age 30 to 50. Sometimes it is linked with lupus. The typical treatment is chemotherapy with a combination of medicines.

Waldenström macroglobulinemia (WM) cells grow mainly in your bone marrow. There they can crowd out normal, healthy blood cells that your body needs (white blood cells, red blood cells and platelets). WM cells also make large amounts of an antibody called immunoglobulin M. This can build up around your body, causing symptoms of its own.

Treatment for WM differs from treatment for other types of lymphoma, and there’s no single standard treatment. It’s important to be treated at a specialized center with WM expertise, where experienced physicians can tailor treatment to you.

Learn More About Waldenström Macroglobulinemia