Extranodal NK-T-cell lymphoma (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2]

Overview

Lymphoma is a type of cancer that originates in lymphocytes. There are many classifications for lymphoma. In common classification, there are two kinds of lymphoma: Hodgkin's disease and Non-Hodgkin lymphoma. Hodgkin's disease is also called Hodgkin's lymphoma.

What are the symptoms of Lymphoma?

• Soaking night sweats
• Swollen, painless lymph nodes in the neck, armpits or groin
• Unexplained weight loss
• Fever
• Coughing, trouble breathing or chest pain
• Weakness and tiredness that don't go away
• Pain, swelling or a feeling of fullness in the abdomen

What causes Lymphoma?

Non-Hodgkin lymphomas begin when a type of white blood cell, called a T cell or B cell, becomes abnormal. The cell divides again and again, making more abnormal cells. These abnormal cells can spread to almost any other part of the body. Most of the time, doctors can't determine why a person gets non-Hodgkin lymphoma.

Who is at highest risk?

• Risk factor is anything that changes your chance of getting a disease like cancer. Different cancers have different risk factors.
• Some risk factors are modifiable and some are nonmodifiable.
• Having the risk factor doesn't always mean that you will get the disease but it certainly increases your chances as compared to the general population.

Risk factors for lymphoma are as under:

Non-Modifiable

• Age
  • Getting older is a strong risk factor for lymphoma overall, with most cases occurring in people in their 60s or older. But some types of lymphoma are more common in younger people.
• Gender
  • Overall, the risk of NHL is higher in men than in women, but there are certain types of NHL that are more common in women. The reasons for this are not known.
• Race, Ethnicity, and Geography
  • In the United States, whites are more likely than African Americans and Asian Americans to develop NHL.
  • Worldwide, NHL is more common in developed countries, with the United States and Europe having some of the highest rates. Some types of lymphoma are linked to certain infections (described further on) that are more common in some parts of the world.
• Family history
  • Having a first degree relative (parent, child, sibling) with NHL increases your risk of developing NHL.

Modifiable

• Exposure to certain chemicals
  • Some studies have suggested that chemicals such as benzene and certain herbicides and insecticides (weed- and insect-killing substances) may be linked to an increased risk of NHL. Research to clarify these possible links is still in progress.
  • Some chemotherapy drugs used to treat other cancers may increase the risk of developing NHL many years later. For example, patients who have been treated for Hodgkin lymphoma have an increased risk of later developing NHL. But it’s not totally clear if this is related to the disease itself or if it is an effect of the treatment.
  • Some studies have suggested that certain drugs used to treat rheumatoid arthritis (RA), such as methotrexate and the tumor necrosis factor (TNF) inhibitors, might increase the risk of NHL. But other studies have not found an increased risk. Determining if these drugs increase risk is complicated by the fact that people with RA, which is an autoimmune disease, already have a higher risk of NHL (see below).
• Radiation exposure
  • Studies of survivors of atomic bombs and nuclear reactor accidents have shown they have an increased risk of developing several types of cancer, including NHL, leukemia, and thyroid cancer.
  • Patients treated with radiation therapy for some other cancers, such as Hodgkin lymphoma, have a slightly increased risk of developing NHL later in life.
  • This risk is greater for patients treated with both radiation therapy and chemotherapy.

• Autoimmmune diseases
  • Some autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE or lupus), Sjogren (Sjögren) disease, celiac disease (gluten-sensitive enteropathy), and others have been linked with an increased risk of NHL.
• Certain infections
  • Epstein-Barr virus (EBV)
  • Human herpesvirus 8 (HHV-8)
  • Human immunodeficiency virus (HIV)
  • Helicobacter pylori
  • Chlamydophila psittaci
  • Campylobacter jejuni
  • Hepatitis C
• Body Weight and Diet
  • Some studies have suggested that being overweight or obese may increase your risk of NHL. Other studies have suggested that a diet high in fat and meats may raise your risk. More research is needed to confirm these findings. In any event, staying at a healthy weight and eating a healthy diet have many known health benefits outside of the possible effect on lymphoma risk.
• Breast implants
  • It is rare, but some women with breast implants develop a type of anaplastic large cell lymphoma (ALCL) in their breast. This seems to be more likely with implants that have textured (rough) surfaces (as opposed to smooth surfaces).

Diagnosis

There are several diagnostic tests used to diagnose lymphoma. It includes

Medical history and Physical examination

• Your doctor will want to get a complete medical history, including information about your symptoms, possible risk factors, and other medical conditions. Next, the doctor will examine you, paying special attention to the lymph nodes and other areas of the body that might be affected, including the spleen and liver. Because infections are the most common cause of enlarged lymph nodes, the doctor will look for an infection near the swollen lymph nodes. The doctor also might order blood tests to look for signs of infection or other problems. Blood tests aren't used to diagnose lymphoma, though. If the doctor suspects that lymphoma might be causing your symptoms, he or she might recommend a biopsy of a swollen lymph node or other affected area.

Biopsy

• For a biopsy, a small piece of a lymph node or, more often, an entire lymph node is removed for testing in a lab. A biopsy is the only way to confirm a person has the NHL. But it's not always done right away because many symptoms of NHL can also be caused by other problems, like an infection, or by other kinds of cancer. For example, enlarged lymph nodes are more often caused by infections than by lymphoma. Because of this, doctors often prescribe antibiotics and wait a few weeks to see if the lymph nodes shrink. If the nodes stay the same or continue to grow, the doctor might order a biopsy. A biopsy might be needed right away if the size, texture, or location of a lymph node or the presence of other symptoms strongly suggests lymphoma.
  • Excisional or incisional biopsy
    • This is the preferred and most common type of biopsy if lymphoma is suspected because it almost always provides enough of a sample to diagnose the exact type of NHL. In this procedure, a surgeon cuts through the skin to remove the lymph node.
  • If the doctor removes the entire lymph node, it is called an excisional biopsy. If a small part of a larger tumor or node is removed, it is called an incisional biopsy.
  • If the enlarged node is just under the skin, this is a fairly simple operation that can often be done with local anesthesia (numbing medicine). But if the node is inside the chest or abdomen, you will be sedated (given drugs to make you drowsy and relaxed) or given general anesthesia (drugs to put you into a deep sleep).
  • Needle biopsy
    • Needle biopsies are less invasive than excisional or incisional biopsies, but the drawback is that they might not remove enough of a sample to diagnose lymphoma (or to determine which type it is). Most doctors do not use needle biopsies to diagnose lymphoma. But if the doctor suspects that your lymph node is enlarged because of an infection or by the spread of cancer from another organ (such as the breast, lungs, or thyroid), a needle biopsy may be the first type of biopsy done. An excisional biopsy might still be needed even after a needle biopsy has been done, to diagnose and classify lymphoma.
    • There are 2 main types of needle biopsies. In a fine needle aspiration (FNA) biopsy, the doctor uses a very thin, hollow needle attached to a syringe to withdraw (aspirate) a small amount of tissue from an enlarged lymph node or a tumor mass. For a core needle biopsy, the doctor uses a larger needle to remove a slightly larger piece of tissue.
    • To biopsy an enlarged node just under the skin, the doctor can aim the needle while feeling the node. If the node or tumor is deep inside the body, the doctor can guide the needle using a computed tomography (CT) scan or ultrasound (see descriptions of imaging tests later in this section).
    • If lymphoma has already been diagnosed, needle biopsies are sometimes used to check abnormal areas in other parts of the body that might be from the lymphoma spreading or coming back after treatment.

Other types of Biopsies

• Bone marrow aspiration and biopsy
  • These procedures are often done after lymphoma has been diagnosed to help determine if it has reached the bone marrow. The 2 tests are often done at the same time. The samples are usually taken from the back of the pelvis (hip) bone, although in some cases they may be taken from other bones.
  • For a bone marrow aspiration, you lie on a table (either on your side or on your belly). After cleaning the skin over the hip, the doctor numbs the area and the surface of the bone with a local anesthetic, which can cause a brief stinging or burning sensation. A thin, hollow needle is then inserted into the bone and a syringe is used to suck out a small amount of liquid bone marrow. Even with the anesthetic, most people still have some brief pain when the marrow is removed.
  • A bone marrow biopsy is usually done just after the aspiration. A small piece of bone and marrow is removed with a slightly larger needle that is pushed into the bone. The biopsy can also cause some brief pain.
• Lumbar puncture (Spinal tap)
  • This test looks for lymphoma cells in the cerebrospinal fluid (CSF), which is the liquid that bathes the brain and spinal cord. Most people with lymphoma will not need this test. But doctors may order it for certain types of lymphoma or if a person has symptoms that suggest the lymphoma may have reached the brain.
  • For this test, you may lie on your side or sit up. The doctor first numbs an area in the lower part of your back over the spine. A small, hollow needle is then placed between the bones of the spine to withdraw some of the fluid.
• Pleural or peritoneal fluid sampling
  • Lymphoma that has spread to the chest or abdomen can cause fluid to build up. Pleural fluid (inside the chest) or peritoneal fluid (inside the abdomen) can be removed by placing a hollow needle through the skin into the chest or abdomen.
  • When this procedure is used to remove fluid from the area around the lung, it’s called a thoracentesis. When it is used to collect fluid from inside the abdomen, it’s known as paracentesis.
  • The doctor uses a local anesthetic to numb the skin before inserting the needle. The fluid is then taken out and checked in the lab for lymphoma cells.

Lab tests on biopsy samples

• Flow cytometry and Immunohistochemistry
  • For both flow cytometry and immunohistochemistry, the biopsy samples are treated with antibodies that stick to certain proteins on cells. The cells are then looked at in the lab (immunohistochemistry) or with a special machine (for flow cytometry), to see if the antibodies attached to them.
  • These tests can help determine whether a lymph node is swollen because of lymphoma, some other cancer, or a non-cancerous disease. The tests can also be used for immunophenotyping – determining which type of lymphoma a person has based on certain proteins in or on the cells. Different types of lymphocytes have different proteins on their surface, which correspond to the type of lymphocyte and how mature it is.
• Chromosome test

Normal human cells have 23 pairs of chromosomes (strands of DNA), each of which is a certain size and looks a certain way in the lab. But in some types of lymphoma, the cells have changes in their chromosomes, such as having too many, too few, or abnormal chromosomes. These changes can often help identify the type of lymphoma.

• Cytogenetics
  • In this lab test, the cells are checked for any abnormalities in the chromosomes.
• Fluorescent in situ hybridization (FISH)
  • This test looks more closely at lymphoma cell DNA using special fluorescent dyes that only attach to specific genes or parts of chromosomes. FISH can find most chromosome changes that can be seen in standard cytogenetic tests, as well as some gene changes too small to be seen with cytogenetic testing. FISH is very accurate and can usually provide results within a couple of days.
• Polymerase chain reaction (PCR)
  • PCR is a very sensitive DNA test that can find gene changes and certain chromosome changes too small to be seen with a microscope, even if very few lymphoma cells are present in a sample.

Imaging tests

• Chest x-ray
  • The chest might be x-rayed to look for enlarged lymph nodes in this area.
• Computed tomography (CT) scan
  • A CT scan combines many x-rays to make detailed, cross-sectional images of your body. This scan can help tell if any lymph nodes or organs in your body are enlarged. CT scans are useful for looking for lymphoma in the abdomen, pelvis, chest, head, and neck. A CT can also be used to guide a biopsy needle into a suspicious area. For this procedure, you lie on the CT scanning table while the doctor moves a biopsy needle through the skin and toward the area. CT scans are repeated until the needle is in the right place. A biopsy sample is then removed to be looked at in the lab.
• Magnetic resonance imaging (MRI) Scan
  • Like CT scans, MRI scans show detailed images of soft tissues in the body. But MRI scans use radio waves and strong magnets instead of x-rays. This test is not used as often as CT scans for lymphoma, but if your doctor is concerned about spread to the spinal cord or brain, MRI is very useful for looking at these areas.
• Ultrasound
  • Ultrasound uses sound waves and their echoes to create pictures of internal organs or masses. In the most common type of ultrasound, a small, microphone-like instrument called a transducer is placed on the skin (which is first lubricated with a gel). It gives off sound waves and picks up the echoes as they bounce off the organs. The echoes are converted by a computer into an image on a computer screen. It can be used to look at lymph nodes near the surface of the body or to look inside your abdomen for enlarged lymph nodes or organs such as the liver and spleen. It can also detect kidneys that have become swollen because the outflow of urine has been blocked by enlarged lymph nodes.
• Positron emission tomography (PET) scan
• For a PET scan, you are injected with a slightly radioactive form of sugar, which collects mainly in cancer cells. A special camera is then used to create a picture of areas of radioactivity in the body. The picture is not detailed like a CT or MRI scan, but it can provide helpful information about your whole body.
• If you have lymphoma, a PET scan might be done to:
  • See if an enlarged lymph node contains lymphoma.
  • Find small areas that might be lymphoma, even if the area looks normal on a CT scan.
  • Check if lymphoma is responding to treatment. Some doctors will repeat the PET scan after 1 or 2 courses of chemotherapy. If the chemotherapy is working, the lymph nodes will no longer absorb the radioactive sugar.
  • Help decide whether an enlarged lymph node still contains lymphoma or is just scar tissue after treatment.
• PET/CT scan: Some machines can do both a PET scan and a CT scan at the same time. This lets the doctor compare areas of higher radioactivity on the PET scan with the more detailed appearance of that area on the CT scan. PET/CT scans can often help pinpoint the areas of lymphoma better than a CT scan alone.
• Bone scan
• This test is usually done if a person is having bone pain or has lab results that suggest the lymphoma may have reached the bones. A radioactive substance called technetium is injected into a vein. It travels to damaged areas of bone, and a special camera can then detect the radioactivity. Lymphoma often causes bone damage, which may be seen on a bone scan. But bone scans can’t show the difference between cancers and non-cancerous problems, such as arthritis and fractures, so further tests might be needed.

Other tests

• Complete blood count
  • A complete blood count (CBC) measures the levels of different cells in the blood. For a person already known to have lymphoma, low blood cell counts might mean that the lymphoma is growing in the bone marrow and affecting new blood cell formation.
• Blood chemistry test
  • Blood chemistry tests are often done to look at how well the kidney and liver function are working.
• Lactate dehydrogenase
  • If lymphoma has been diagnosed, the lactate dehydrogenase (LDH) level may be checked. LDH levels are often increased in patients with lymphomas.

Test of heart and lung function

• These tests are not used to diagnose lymphoma, but they might be done if you are going to get certain chemotherapy drugs commonly used to treat lymphoma that could affect the heart or the lungs. Your heart function may be checked with an echocardiogram (an ultrasound of the heart) or a MUGA scan. Your lung function may be checked with pulmonary function tests, in which you breathe into a tube connected to a machine.

Treatment options

There are two types of lymphoma Hodgkin's and Non-Hodgkin's. Treatment options for both of them are as under:

Hodgkin's lymphoma

• Early Favorable Hodgkin Lymphoma
  • Treatment of early favorable Hodgkin lymphoma may include:
    • Combination chemotherapy.
    • Combination chemotherapy with radiation therapy to parts of the body with cancer.
    • Radiation therapy alone to areas of the body with cancer or to the mantle field (neck, chest, armpits).
• Early Unfavorable Hodgkin Lymphoma
  • Treatment options for early unfavorable Hodgkin lymphoma may include:
    • Combination chemotherapy.
    • Combination chemotherapy with radiation therapy to parts of the body with cancer.
• Advanced Hodgkin Lymphoma
  • Treatment options for advanced Hodgkin lymphoma may include:
    • Combination chemotherapy.
• Recurrent Adult Hodgkin Lymphoma
  • Treatment options for recurrent Hodgkin lymphoma may include:
    • Combination chemotherapy.
    • Combination chemotherapy followed by high-dose chemotherapy and stem cell transplant with or without radiation therapy.
    • Combination chemotherapy with radiation therapy to parts of the body with cancer in patients older than 60 years.
    • Radiation therapy with or without chemotherapy.
    • Chemotherapy as palliative therapy to relieve symptoms and improve quality of life.
      • A clinical trial of high-dose chemotherapy and stem cell transplant.
      • A clinical trial of lower-dose chemotherapy and radiation therapy followed by stem cell transplant.
      • A clinical trial of a monoclonal antibody.
      • A clinical trial of chemotherapy.

Non-Hodkin's lymphoma

• Aggressive stage I and aggressive, contiguous stage II adult non-Hodgkin lymphoma
  • Monoclonal antibody therapy and combination chemotherapy. Sometimes radiation therapy is given later.
  • A clinical trial of a new regimen of monoclonal antibody therapy and combination chemotherapy.
• Aggressive, noncontiguous stage II, III, or IV adult non-Hodgkin lymphoma
  • Monoclonal antibody therapy with combination chemotherapy.
  • Combination chemotherapy.
  • A clinical trial of monoclonal antibody therapy with combination chemotherapy followed by radiation therapy.

Other treatments depend on the type of aggressive non-Hodgkin lymphoma. Treatment may include the following:

• For extranodal NK -/T-cell lymphoma, radiation therapy that may be given before, during, or after chemotherapy and CNS prophylaxis.
• For mantle cell lymphoma, monoclonal antibody therapy with combination chemotherapy, followed by stem cell transplant. Monoclonal antibody therapy may be given afterwards as maintenance therapy (treatment that is given after initial therapy to help keep cancer from coming back).
• For posttransplantation lymphoproliferative disorder, treatment with immunosuppressive drugs may be stopped. If this does not work or cannot be done, monoclonal antibody therapy alone or with chemotherapy may be given. For cancer that has not spread, surgery to remove the cancer or radiation therapy may be used.
• For plasmablastic lymphoma, treatments are like those used for lymphoblastic lymphoma or Burkitt lymphoma.

For information on the treatment of lymphoblastic lymphoma, see Treatment Options for Lymphoblastic Lymphoma and for information on the treatment of Burkitt lymphoma, see Treatment Options for Burkitt Lymphoma.

Where to find medical care for Lymphoma?

Directions to Hospitals Treating Lymphoma

Source

https://www.cancer.org/cancer/non-hodgkin-lymphoma/causes-risks-prevention/risk-factors.html

https://www.cancer.org/cancer/non-hodgkin-lymphoma/detection-diagnosis-staging/how-diagnosed.html

https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq#section/_419

https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq#section/_86

Related chapters

• Hodgkin's lymphoma (patient information)
• Non-Hodgkin lymphoma (patient information)
• B-cell lymphoma (patient information)
• T-cell lymphoma (patient information)