Also Known As
Hodgkin Lymphoma
HL
Hodgkin Disease
Non-Hodgkin Lymphoma
NHL
Lymphoproliferative Disorder
This article was last reviewed on
This article waslast modified on January 26, 2021.
What is lymphoma?

Lymphoma is cancer that often develops within one or more lymph nodes in the lymphatic system. It starts when white blood cells called lymphocytes grow and divide uncontrollably.

Lymphocytes are an important part of the body's natural defense (immune) system. They help to protect against infections, among other functions. Lymphocytes circulate throughout the body in both the blood and the lymphatic system.

The lymphatic system is a network of lymph nodes and vessels (lymphatics) that drain fluids from the body's tissues and carry them as "lymph" through the body and back to the bloodstream.

Lymph nodes are found singly and in chains along the lymphatic vessels, in areas that include the neck, armpits, chest, abdomen, and groin. The nodes filter lymph fluid, destroying any microbes and abnormal cells that may be present. Lymph nodes contain different types of lymphocytes:

  • T-lymphocytes help control the immune system. They initiate the immune response, control how big or small it should be, and shut it down when it is not needed. In addition, they recognize foreign substances and process them for removal.
  • B-lymphocytes make antibodies. Antibodies are proteins produced in response to infections and help fight infections, such as measles, mumps, or hepatitis.
  • Natural killer (NK) cells make up about 10-15% of total lymphocytes in the blood. NK cells attack and "kill" abnormal cells such as cancer cells or those infected with viruses.

Any one of these cells or a combination of them can be involved in lymphoma. As they grow out of control, abnormal lymphocytes can begin to outnumber normal cells in the lymph node, leading to an enlarged lymph node. The abnormal cells can eventually spread (metastasize) to one or more other lymph nodes and related organs, including the spleen, bone marrow, tonsils, adenoids, and thymus. Besides lymph nodes, lymphoma can also develop in various other tissues and internal organs such as the stomach, intestine, and brain.

There are two main types of lymphoma. B-cell lymphomas are more common than T-cell lymphomas. NK cell lymphoma is very rare.

In addition to cell type, lymphomas are classified based on the lymphoma cell characteristics, and how fast or slow the lymphomas grow and spread (metastasize). The World Health Organization (WHO) has classified many different types of lymphoma. Some examples are provided in the next two sections. For a full list, see The 2016 revision of the World Health Organization classification of lymphoid neoplasms.

Accordion Title
About Lymphoma
  • Hodgkin Lymphoma (Hodgkin Disease)

    There are different types of Hodgkin lymphoma (also called HL or Hodgkin's disease) that grow and spread differently. The two main types of Hodgkin lymphoma in adults are classic (cHL) and nodular lymphocyte predominant.

    • About 95% of cases of Hodgkin lymphoma are the classic type. Classic Hodgkin lymphoma is marked by the presence of large, cancerous cells called Reed-Sternberg cells. These cells are usually an abnormal type of B lymphocyte. Classic Hodgkin lymphoma is grouped into 4 subtypes based on the location of affected lymph nodes and how those nodes look under a microscope.
    • The nodular lymphocyte predominant type makes up about 5% of Hodgkin lymphomas. The cancerous lymphoma cells are often very big and have a "popcorn"-shaped nucleus (so called "popcorn cells"). This type is most common in younger people. It tends to grow more slowly and is treated differently than the classic type.

    Hodgkin lymphoma can affect people of any age, but it is most common in young adults in their 20's and people over age 55. It is slightly more common in males than females. According to the American Cancer Society, about 8,500 people in the United States are diagnosed with Hodgkin lymphoma each year and about 970 people die of it.

    Hodgkin lymphoma is caused by a change (mutation) in the DNA of a lymphocyte. Experts don't yet know what causes the DNA changes in lymphocytes that make them turn into Reed-Sternberg cells. Some scientists think that a reaction to an infection with Epstein-Barr virus (the virus that causes "mono") can cause a mutation in B lymphocytes and this sometimes leads to the development of Reed-Sternberg cells. Other infections may be the trigger that lead to Reed-Sternberg cells in some cases. More research is needed to determine the cause of Hodgkin lymphoma.

  • Non-Hodgkin Lymphoma

    According to the American Cancer Society, non-Hodgkin lymphoma (NHL) accounts for about 4% of all cancers. About 74,000 people are diagnosed with non-Hodgkin lymphoma per year and about 20,000 people die from it. The cellular composition seen under microscope and clinical behavior of NHL are very different from that of Hodgkin lymphoma.

    • NHL can occur at any age, but the risk grows greater as you get older. More than half of people diagnosed with NHL are age 65 or older.
    • The overall risk for NHL is slightly greater in men than women, but there are some types of NHL that are more common in women.
    • In the U.S., whites (Caucasians) are more likely than African Americans or Asian Americans to develop NHL.

    Experts believe the DNA (gene and/or chromosome) changes in lymphocytes that lead to NHL usually happen during a person's lifetime, although in some cases, they are inherited. Exposure to radiation, certain chemicals or drugs, and certain infections can increase risk of developing NHL. People with weakened immune systems and autoimmune disorders (such as rheumatoid arthritis or lupus) may also be at increased risk for NHL.

    In general, non-Hodgkin lymphomas are classified by cell type and characteristics, and how fast or slow they grow and spread (metastasize). The World Health Organization has classified over 80 different types of NHL. In the United States, B-cell non-Hodgkin lymphomas are much more common than T-cell and NK-cell lymphomas.

    About 85 to 90% of non-Hodgkin lymphomas involve mature B-lymphocytes. Examples of some of these include:

    • Diffuse large B-cell lymphoma (DLBCL): this is the most common type in the U.S., accounting for about one in three cases of all non-Hodgkin lymphomas. It is a fast-growing lymphoma and can affect anyone of any age but occurs mostly in older people.
    • Follicular lymphomas: these make up about one in five of all lymphomas in the U.S. This is generally a very slow-growing lymphoma, but over time some transform into fast-growing DLBCL.
    • Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL / SLL): These are two closely related diseases. The difference between them is that CLL mainly affects the bone marrow and blood, while SLL is found mainly in the lymph nodes. These are usually slow-growing diseases, and the treatment for both is the same.
    • Waldenstrom macroglobulinemia (WM): in this condition, cancerous B cells grow mainly in the bone marrow, but they can also grow in the liver, spleen, and lymph nodes. The cancer cells make excess amounts of big protein (immunoglobulin of IgM type) that builds up in the blood, causing it to thicken. WM is a slow-growing lymphoma that typically affects people in their 60s.

    Some other examples include:

    • Marginal zone lymphoma (MZL)
    • Mantle cell lymphoma (MCL)
    • Gastric mucosa-associated lymphoid tissue (MALT) lymphoma
    • Lymphoblastic lymphoma (LPL) (LPL can progress to WM)
    • Burkitt lymphoma (BL)
    • Primary mediastinal (thymic) large B-cell lymphoma (PMBCL)
    • Hairy cell leukemia

    About 10 to 15% of non-Hodgkin lymphomas affect mature T-lymphocytes and NK-cells. Examples of some of these include:

    • T-lymphoblastic lymphoma (leukemia): this type can be considered either a lymphoma or a type of acute lymphoblastic leukemia depending on how much of the bone marrow is affected. About 1% of all lymphomas are this type. It is most common in teens and young adults and affects males more than females.
    • Peripheral T-cell lymphomas (PTCL) not otherwise specified: there are many kinds of mature T-cell lymphomas that don't fit into any of the defined categories. All together, they make up about 4% to 5% of all lymphomas.
    • Cutaneous T-cell lymphomas (mycosis fungoides, Sezary syndrome, and others): these are less common forms of lymphoma. While most lymphomas begin in lymphoid tissue or internal organs, these types start in the skin. Skin lymphomas make up about 4% of all lymphomas.

    A few other examples include:

    • Systemic anaplastic large-cell lymphoma (ALCL)
    • Adult T-cell leukemia/lymphoma
    • Angioimmunoblastic T-cell lymphoma (AITL)
  • Signs and Symptoms

    Lymphoma can sometimes be difficult to diagnose because the signs and symptoms are often mild. You may not have any signs or symptoms, especially at first, but may develop some symptoms if the lymphoma grows larger. Usually there are swollen lymph nodes, but the node may not be visible or felt by you or your healthcare provider.

    Some of the main signs and symptoms of lymphoma include:

    • Painless swelling of one or more lymph nodes in the armpits, neck, or groin; other conditions can cause swollen lymph nodes, including infections, but these are usually painful when touched.
    • Enlarged spleen
    • An increase in abnormal lymphocytes in the blood

    Other common signs and symptoms may include:

    • Fever without an infection (it may come and go over several weeks)
    • Night sweats
    • Unexplained weight loss (as much as 10% of body weight or more)
    • Frequent and/or serious infections
    • Easy bruising, bleeding

    There may be various other general signs and symptoms present, but many of these can also be caused by other conditions. Some examples include:

    • Fatigue (extreme tiredness)
    • Loss of appetite
    • Itchiness
    • Neck or flank pain

    Depending on where the lymphoma is in your body, it can cause other serious signs and symptoms:

    • If the lymphoma is in a lymph node in your chest, it may cause chest pain or pressure. It may affect your breathing, causing shortness of breath or cough.
    • If it is in your abdomen, it can cause a swollen abdomen and /or abdominal pain.
  • Testing

    Your healthcare practitioner will perform a physical exam and find out about your medical history. Additionally, a number of laboratory tests may be used to help diagnose lymphoma, determine the exact type, and monitor the effectiveness of treatment. After successful treatment (remission), testing may be used to check whether lymphoma has returned.

    Laboratory tests

    A biopsy of affected lymph nodes or lymphoid tissue is the gold standard diagnostic test for both Hodgkin lymphoma and non-Hodgkin lymphoma. A pathologist uses a microscope to examine samples of lymph tissue to determine whether cancer is present. The sample is usually collected during surgery or by a fine needle aspiration or biopsy procedure. Immunohistochemistry can be performed on tissue biopsy to help diagnose and classify lymphoma. (To learn more about biopsies, read the article on Anatomic Pathology.)

    Several other laboratory tests that may be used to help diagnose and monitor lymphoma as well as any complications.

    General laboratory tests may include:

    • Complete blood count (CBC) – this panel of tests evaluates white blood cells, red blood cells, and platelets. It may be used to rule out non-lymphoma conditions (such as leukemia) and/or to see if anemia is present. A CBC can determine if the platelet count and/or white blood cell count are low, which may indicate that lymphoma is present in the bone marrow and/or blood.
    • Bone marrow biopsy and examination – used to evaluate the cells present in the bone marrow. Abnormal lymphocytes can be seen in the bone marrow involved by lymphoma.
    • Blood smear – a microscope is used to evaluate red blood cells, white blood cells, and platelets as well as any abnormal cells (e.g., lymphoma cells) that may be present.

    Selective laboratory tests may include:

    Non-laboratory tests
    Examples of some imaging tests that may be performed include:

    • Computed tomography (CT) scans
    • Positron emission tomography (PET) scan
    • Chest X-ray
    • Magnetic resonance imaging (MRI)

    For more on these imaging studies, visit RadiologyInfo.org.

  • Staging

    Because more than one lymph node can be involved, it is important to find out which ones are affected and where they are in the body. This process is called staging. The following table contains an example of a classification system that describes how widespread the disease is. The Ann Arbor staging system is most used for non-Hodgkin lymphoma in adults.

    Stages Found in Lymphomas

    Stage Description
    I Stage I occurs when there is a single lymphoid area (such as a lymph node), a pair of areas (tonsils), or a group of related areas (the tonsils and adenoids) involved. It also occurs when cancer is found only in one area of a single organ outside of the lymph system.
    II Stage II occurs when there are two or more lymphoid areas involved, but they are on the same side of the diaphragm (the muscle under the rib cage that controls breathing and separates the chest and abdomen). An example of this would be the tonsils and one underarm lymph node. It also occurs when the lymphoma extends from a single lymph node group into a nearby organ, or when it affects groups of lymph nodes on the same side of the diaphragm.
    III Stage III occurs when there is involvement on both sides of the diaphragm (above and below), such as a node in the neck and another in the abdomen. It also occurs if the cancer has spread into an area or organ next to the lymph nodes, into the spleen, or both.
    IV Stage IV has involvement throughout the body and, in particular, in major sites such as the bone marrow and/or liver.

    Staging can help guide therapy. For example, if you have Stage I lymphoma with only one node involved, then surgically removing that node may result in a cure. On the other hand, if you have Stage IV lymphoma, then surgical removal is not generally possible.

    The most commonly used staging tests performed in the clinical laboratory are the complete blood count (CBC), liver and kidney function studies, and bone marrow biopsy. Non-laboratory tests include CT scans, MRI evaluations, and X-ray procedures.

  • Treatment

    Each lymphoma is unique. Some are more likely to grow and spread (aggressive) than others. The healthcare professional will "grade" the cancer based on its apparent aggressiveness. This information, along with the type and location(s) of the lymphoma, are used to guide treatment. Some people without troublesome or serious symptoms may be monitored closely and only treated when symptoms worsen.

    The ultimate goal of treatment is a cure. When a cure is not possible, the goals are to put the lymphoma into remission (no signs of disease) for as long as possible, slow or prevent the disease from getting worse (progressing), relieve any symptoms, and prevent and treat any complications.

    Lymphoma treatment often uses a combination of approaches. This may include:

    • Chemotherapy
    • Radiation therapy
    • High-dose chemotherapy followed by stem cell transplantation
    • Targeted therapy—these are drugs that target the specific changes in lymphoma cells that make them grow out of control.
    • Immunotherapy—this is treatment that may help boost your immune system to fight the cancer or is man-made immune system parts that can help kill or slow the cancer.
    • Surgery—this is rarely used to remove organs affected by lymphoma.

    The choice of treatments and the outlook for your disease (prognosis) depends on the type, stage, and grade of the disease and your general health. Hodgkin lymphoma is mainly treated with chemotherapy and radiation therapy. One or more of the other options might be used as well.

    All people diagnosed with lymphoma, even those whose lymphoma has been "cured" or put into remission, must be monitored for the rest of their lives. Many never experience lymphoma again but in others, it may come back years later.

    For more details on specific treatments, see the links in the Related Content section.

View Sources

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