Primary cutaneous follicle centre lymphoma (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Preeti Singh, M.B.B.S.[2] Sowminya Arikapudi, M.B,B.S. [3] Soroush Seifirad, M.D.[4]


There are two kinds of lymphoma: Hodgkin's disease and Non-Hodgkin lymphoma. Non-Hodgkin lymphoma is cancer of the lymphoid tissue, which includes the lymph nodes, spleen, and other organs of the immune system. Non-Hodgkin lymphoma is common in America. Usual symptoms include unexplained weight loss, fever, drenching night sweat, etc. Non-Hodgkin lymphoma can be diagnosed by biopsy. Treatment includes radiation therapy, chemotherapy, bone marrow or peripheral blood stem cell transplantation and a combination of them.

What are the symptoms of Non-Hodgkin lymphoma?

Early Non-Hodgkin lymphoma does not have any symptoms. People may notice one or more of the following symptoms as the tumor developing or when detected by regular examinations:

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

Coughing or shortness of breath may occur if the cancer affects the thymus gland or lymph nodes in the chest, which may put pressure on the windpipe (trachea) or other airways.

Some patients may have abdominal pain or swelling, which may lead to a loss of appetite, constipation, nausea, and vomiting.

If the cancer affects cells in the brain, the person may have a headache, concentration problems, personality changes, or seizures.

What causes Non-Hodgkin lymphoma?

White blood cells called lymphocytes are found in lymph tissues. They help prevent infections. Most lymphomas start in a type of white blood cells called B lymphocytes, or B cells.

For most patients, the cause of this cancer is unknown. However, lymphomas may develop in people with weakened immune systems. For example, the risk of lymphoma increases after an organ transplant or in people with HIV infection.

There are many different types of non-Hodgkin's lymphoma (NHL). It is grouped according to how fast cancer spreads.

  • cancer may be low grade (slow-growing), intermediate grade, or high grade (fast-growing). Burkitt's lymphoma is an example of a high-grade lymphoma. Follicular lymphoma is a low-grade lymphoma.
  • The cancer is further grouped by how the cells look under the microscope, for example, if there are certain proteins or genetic markers present.

According to the American Cancer Society, a person has a 1 in 50 chance of developing non-Hodgkin's lymphoma. NHL most often affects adults. However, children can get some forms of lymphoma. You are more likely to get lymphoma if you have a weakened immune system or have had an organ transplant.

This type of cancer is slightly more common in men than in women.

Who is at highest risk?

Clinical data has suggested that the development of Non-Hodgkin lymphoma is related to several factors.

  • Immune system deficiency: People with weakened immune systems, such as people who receive organ transplants or infected with HIV, have an increased risk for non-Hodgkin lymphoma.
  • Autoimmune diseases: Some autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE), and others have been linked with an increased rate of non-Hodgkin lymphoma.
  • Certain infections such as T-cell leukemia/lymphoma virus (HTLV-1), Epstein-Barr virus (EBV)
  • Exposure of chemicals such as benzene, certain herbicides, and insecticides, exposure to radiation
  • Bad life-style: Overweight, obese, diet poor in vegetables.
  • Age: Most cases of Non-Hodgkin lymphoma occur in people older than 60.
  • Gender: Non-Hodgkin lymphoma occurs slightly more often in men than in women.
  • Geography: Worldwide, non-Hodgkin lymphoma is more common in developed countries, with the United States and Europe having the highest rates.

When to seek urgent medical care?

Call your health care provider if symptoms of Non-Hodgkin lymphoma develop. If you experience either of the following symptoms, seeking urgent medical care as soon as possible:


  • Lymph nodes biopsy and a series of tests on samples: Many of the symptoms of Non-Hodgkin lymphoma are not specific enough to say for certain. Other health problems may also cause these symptoms. So biopsy on lymph nodes may be the most valuable test. The goal of a biopsy is to get enough of a sample to identify the diagnosis, as well as the type of the disease. Usual types of biopsy include excisional or incisional biopsy, fine needle aspiration (FNA) or core needle biopsy and bone marrow aspiration and biopsy. After checking under the microscope by the pathologists, sometimes the samples need to further test by a series of tests such as immunohistochemistry, flow cytometry, fluorescent in situ hybridization (FISH) and so on.
  • Images tests such as computed tomography (CT) scan, magnetic resonance imaging (MRI), positron emission tomography (PET) scan, ultrasound, chest-x-ray and bone scan: The goal of these image tests are to help find out the extent of the Non-Hodgkin lymphoma and determine the stage of the disease.

Treatment options

Patients with Non-Hodgkin lymphoma have many treatment options. The selection depends on the stage of the disease. The options are radiation therapy, chemotherapy, immunotherapy, and bone marrow or peripheral blood stem cell transplantation. Before treatment starts, ask your health care team about possible side effects and how treatment may change your normal activities. Because cancer treatments often damage healthy cells and tissues, side effects are common. Side effect may not be the same for each person, and they may change from one treatment session to the next.

  • Chemotherapy: This treatment is to use drugs to stop the growth of cancer cells either by killing the cells or by stopping them from dividing. Doctors give chemotherapy in cycles and each chemotherapy cycle generally lasts for several weeks. One of the most common combinations of drugs is called CHOP, which includes the drugs cyclophosphamide (Cytoxan), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone.
  • Radiation therapy: This is a cancer treatment to kill cancer cells or keep them from growing by using high-energy x-rays or other types of radiation. External beam radiation is often used to treat Non-Hodgkin lymphoma.
  • Immunotherapy: Immunotherapies use man-made versions of substances to kill lymphoma cells, slow their growth, or activate the patient's own immune system to more effectively fight the lymphoma.
  • Bone marrow or peripheral blood stem cell transplantation: This treatment allows doctors to give an infusion of blood-forming stem cells after destroying the patient's bone marrow with high-dose chemotherapy. Bone marrow transplants were more common in the past, but they have largely been replaced by peripheral blood stem cell transplant.

Diseases with similar symptoms

Other health problems may also cause similar symptoms. Go to see your doctor verify your diseases as early as possible. Diseases with similar symptoms are listed in the following:

Where to find medical care for Non-Hodgkin lymphoma?

Directions to Hospitals Treating Non-Hodgkin lymphoma

Prevention of Non-Hodgkin lymphoma

Although the reasons for the development of Non-Hodgkin lymphoma are not clear, preventing known risk factors such as immune deficiency may be helpful.

  • Prevention of the immunodeficiency virus (HIV) infection
  • Prevention of T-cell leukemia/lymphoma virus (HTLV-1) infection

What to expect (Outlook/Prognosis)?

The International Prognostic Index (IPI) is the most widely used prognostic system for Non-Hodgkin lymphoma. This system uses 5 factors:

  • Age
  • Lactate dehydrogenase (LDH) level
  • Performance status
  • Clinical stage
  • Sites of extranodal disease

Possible complications

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