Non-Hodgkin Lymphoma Facts

Understanding Non-Hodgkin Lymphoma

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL)

CLL and SLL are closely related. They 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 CLL and SLL tend to be slow-growing, but each patient’s version of these conditions can vary significantly. You and your physician may decide to monitor the disease, but not to treat it. This is called watchful waiting. If you do need active treatment, it could include oral therapy, chemotherapy, antibody therapy, blood or marrow transplant or clinical trial therapy. 

Learn more about CLL

Cutaneous B-cell Lymphoma (CBCL)

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

It is nearly always slow-growing. Almost half of people with this disease have a recurrence after responding to treatment at first. 

Treatment may focus on: 

• The skin. This includes surgery, radiation therapy, phototherapy (using ultraviolet, or UV, light) and medicines that are put on the skin. 
• The whole body (systemic treatments). This includes photopheresis, interferon, targeted therapies, chemotherapy or other medicines, and blood or marrow transplant. 

Learn more about CBCL

Diffuse Large B-cell Lymphoma (DLBCL)

DLBCL is the most common subtype of NHL. It is also a subtype of B-cell NHL. DLBCL makes up about 30 percent of NHL cases in the U.S.  

DLBCL advances quickly. Treatment usually starts right away with chemotherapy and the antibody therapy called rituximab. After the first treatment, about two-thirds of patients will go into long-term remission. In some people, the disease does not respond to treatment (refractory disease), or it gets better but then gets worse (relapsed disease). Then, options include high-dose chemotherapy plus a blood or marrow transplant or cellular immunotherapies, such as axicabtagene ciloleucel (Yescarta®), tisagenlecleucel (Kymriah®) and lisocabtagene maraleucel (Breyanzi®).  

Follicular Lymphoma

Follicular means the cells tend to come from a central part (B-cell follicle) of the lymph node. 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 turns into aggressive diffuse B-cell lymphoma. 

Since it typically grows slowly, you and your physician may decide to monitor the disease, but not treat it. This is known as watchful waiting. 

If you do need active treatment, it could include chemotherapy, radiation therapy or immunotherapy. Many patients go into long-term remission after treatment. For most people, the disease gets better but then gets worse (relapsed disease) or does not respond to treatment (refractory disease). Then, options include cellular immunotherapies, such as axicabtagene ciloleucel (Yescarta®) and lisocabtagene maraleucel (Breyanzi®); high-dose chemotherapy plus a blood or marrow transplant; or treatments that are being tested in clinical trials.

Hairy Cell Leukemia

This is a subtype of B-cell NHL that gets its name from the way the cells look. They have very thin structures on their surface that make them look hairy. The disease is rare, with fewer than 1,000 people diagnosed in the U.S. 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. 

Lymphomatoid Granulomatosis

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 a low impact on your health, you and your physician may decide to monitor the disease, but not treat it. This is called watchful waiting. If you do need active treatment, options include interferon, corticosteroids, chemotherapy and the antibody therapy called rituximab. 

Mantle Cell Lymphoma (MCL)

MCL typically affects men between the ages of 50 and 70. By the time it is diagnosed, it has often already spread throughout the body. 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, and most patients will develop symptoms in weeks to months. 

Treatment may include chemotherapy combined with immunotherapy or clinical trial therapy. Brexucabtagene autoleucel (Tecartus®), available at SCCA, is an FDA-approved cellular immunotherapy treatment for patients with relapsed (the disease gets better but then gets worse) or refractory (does not respond to treatment) MCL. Blood or marrow transplant is an option for some people. Many treatments are being studied in clinical trials. 

Marginal Zone B-cell Lymphoma

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 usually the first treatment, and they often put the cancer into complete remission. If the cancer relapses (gets better but then gets worse), 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. 

Primary Central Nervous System (CNS) Lymphoma

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 from other lymphomas. Symptoms may include headaches, confusion, vision problems, facial paralysis and seizures. 

Patients with this subtype may start treatment by taking corticosteroids to control symptoms and reduce swelling. Other treatments include chemotherapy, radiation therapy and sometimes a blood or marrow transplant. 

Waldenström Macroglobulinemia (Lymphoplasmacytic Lymphoma)

Waldenström macroglobulinemia (WM) cells grow mainly in your bone marrow. There, they can crowd out the 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 other symptoms. 

Treatment for WM is different from treatment for other types of lymphoma, and there is no one standard treatment. It is important to be treated at a specialized center, where physicians who are experts in WM can match your treatment to your situation. 

Learn more about Waldenström macroglobulinemia

Anaplastic Large-cell Lymphoma (ALCL)

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, a body-wide disease that expresses a protein called anaplastic lymphoma kinase (ALK). It usually affects children. 
• ALK-negative (ALK-) ALCL, a body-wide disease that does not 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. 

Angioimmunoblastic T-cell Lymphoma (AITL)

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 get infections. The causes of AITL are unknown, but it has been linked with Epstein-Barr virus. To start, treatment usually involves steroids. It often progresses and may be treated with the standard T-cell NHL treatments — chemotherapy or other medicine-based options  — as well as radiation therapy.

Extranodal NK/T-cell Lymphoma, Nasal Type

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. 

Enteropathy-Type T-cell Lymphoma

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 damaged part of the intestine, chemotherapy and a blood or marrow transplant. A gluten-free diet might prevent this lymphoma. 

Hepatosplenic T-cell Lymphoma

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 because of 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 blood or marrow transplant using a donor’s stem cells (allogeneic transplant) the first time a patient goes into remission. 

Subcutaneous Panniculitis-like T-cell Lymphoma

People with this rare, aggressive disease usually have small bumps or lesions under their skin. It is most common in people age 30 to 50. Sometimes it is linked with lupus. The standard treatment is chemotherapy combined with other medicines. 

Peripheral T-cell Lymphoma, Not Otherwise Specified

Physicians use this name for T-cell lymphomas that do not 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 patients are treated with chemotherapy. 

Aggressive NK/T-cell Lekemia/Lymphoma

This disease affects NK cells and causes skin lesions. It is rare in the U.S. and more common in Asia and parts of Latin America. It usually happens to older adults and is linked with Epstein-Barr virus. The main treatment is chemotherapy and sometimes radiation therapy. Because this condition is likely to relapse (get better but then get worse), clinical trials or referral for blood or bone marrow transplant are often considered. 

Resources

There are many resources online for learning about your disease. We've compiled a list of trusted sources to help you get started.

If you or yur caregiver are seeking additional information and resources, Fred Hutch Patient and Family Resource Center is available to help connect you with what you need. 

Learning About Non-Hodgkin Lymphoma

Our list of online resources provides accurate health information from reliable and reputable sources, like the National Cancer Institute (NCI) and the American Society of Clinical Oncology (ASCO).