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Non-Hodgkin lymphoma

Non-Hodgkin lymphoma types

Non-Hodgkin lymphoma is divided into more than 30 types, classified based on the type of lymphocyte involved: B lymphocytes (B cells) or T lymphocytes (T cells). Non-Hodgkin lymphoma is further classified by other factors, including whether it is aggressive (fast-growing) or indolent (slow-growing).

Aggressive lymphomas include:

  • Diffuse large B-cell lymphoma
  • Anaplastic large-cell lymphoma
  • Burkitt lymphoma
  • Lymphoblastic lymphoma
  • Mantle cell lymphoma
  • Peripheral T-cell lymphoma

Indolent lymphomas include:

  • Follicular lymphoma
  • Cutaneous T-cell lymphoma
  • Lymphoplasmacytic lymphoma
  • Marginal zone B-cell lymphoma
  • MALT lymphoma
  • Small-cell lymphocytic lymphoma

Burkitt lymphoma

Burkitt lymphoma is considered the most aggressive form of lymphoma and is one of the fastest growing of all cancers. But it is very rare, accounting for about 2 percent of all lymphoma diagnoses. The disease originates in mature B-lymphocytes, which are cells of the acquired immune system that produce antibodies to help fight off disease. Burkitt lymphoma, a type of non-Hodgkin lymphoma, is most often diagnosed in young adults and children, especially male. But certain types of Burkitt lymphoma have been diagnosed in adults, especially those with a weakened immune system. The disease is named for Denis Burkitt, the British surgeon who first identified the cancer in African children in the late 1950s.

The symptoms of Burkitt lymphoma vary greatly and depend on the disease’s type. Symptoms may spread or worsen quickly as this aggressive cancer advances. Patients with endemic Burkitt lymphoma may have swelling or disfigurement in the jaw or face. Patients with sporadic Burkitt lymphoma may have swelling or pain in the abdomen. The disease may also spread quickly to the central nervous system and brain, causing severe neurological symptoms, including paralysis.

Other symptoms include:

  • Swollen lymph nodes
  • Night sweats
  • Fever
  • Fatigue
  • Loss of appetite
  • Weight loss

A biopsy, usually of an infected lymph node, is required for an accurate diagnosis if Burkitt lymphoma is suspected.

Lymphoma and leukemia

Lymphoma and leukemia share a common origin—lymphocytes, the white blood cells that originate in the bone marrow. B cells mature in the bone marrow, while T cells mature in the thymus. These cells, which are critical soldiers in the immune system, travel through the lymphatic system and bloodstream and fight off infection and disease. Some types of blood cancer may be considered either leukemia or non-Hodgkin lymphoma, depending on where the cancer originates, as well as other factors. These types of cancer include:

  • Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL): These cancers affect the same cells, small lymphocytes, and are often considered different versions of the same disease. If the cancerous lymphocytes are found in the bone marrow or blood, it is considered CLL, a typically slow-growing cancer. If the cells are located in the lymph nodes or spleen, it is considered SLL. About 5 percent of all non-Hodgkin lymphoma cases are diagnosed as SLL.
  • Precursor T-lymphoblastic leukemia/lymphoma: This form of non-Hodgkin lymphoma, similar to acute lymphocytic leukemia (ALL), is often found in the thymus. This cancer occurs when immature T cells mutate and grow rapidly and may form tumors.
  • Adult T-cell lymphoma/leukemia (ATLL): This type of cancer is most often caused by the human T-cell lymphotropic virus type I (HTLV-1). ATLL that is found in blood is considered leukemia. If it is found in lymph nodes, it is considered lymphoma. ATLL may be aggressive and often affects bones and skin.
  • Hairy cell leukemia: This slow-growing form of leukemia is sometimes considered a type of non-Hodgkin lymphoma. Hairy cell leukemia is very rare, affecting only about 700 patients a year.

B-cell lymphomas

Nearly 90 percent of non-Hodgkin lymphoma types develop in B cells. Common forms of B-cell non-Hodgkin lymphoma include:

  • Diffuse large B-cell lymphoma (DLBCL): The most common type of non-Hodgkin lymphoma in the United States, DLBCL accounts for about 30 percent of all cases. It is most often diagnosed in older adults. DLBCL is aggressive and may spread quickly. It may be found not only in the lymph system, but as a primary disease elsewhere in the body, including the gastrointestinal tract, testes, breasts and brain.
    • Subtypes of DLBCL include:
      • Primary mediastinal B-cell lymphoma is often found in the mediastinum, the area of the chest cavity between the lungs. This type of lymphoma, which is more common in young women, may produce fast-growing tumors that may encompass the mediastinum cavity and press on the airways and blood vessels.
      • Primary central nervous system (CNS) lymphoma originates in the brain or spinal cord and may be found in patients with acquired immunodeficiency syndrome (AIDS) or those whose immune systems may be compromised, including organ transplant patients. When primary CNS lymphoma originates in the eye, it is called ocular lymphoma.
    • Symptoms of DLBCL may include swelling caused by enlarged lymph nodes in the neck, underarms or groin. The swelling may or may not be painless. Other symptoms may include coughing or difficulty swallowing, night sweats, fever, swollen face, weight loss and fatigue.
    • Treatments for DLBCL may include chemotherapy, targeted therapy and sometimes radiation therapy. Surgery may be required in some cases to remove obstructive tumors.
  • Follicular lymphoma: The most common type of indolent, or slow-growing, lymphoma, follicular lymphoma accounts for about 20 percent of all lymphoma cases in the United States. The disease is typically diagnosed in middle-aged or older adults and is most often found in the bone marrow or lymph nodes.
    • Common symptoms of follicular lymphoma include swollen lymph nodes, fatigue, shortness of breath, night sweats and weight loss. In some cases, follicular lymphoma grows so slowly that the patient may not experience obvious symptoms and surveillance or monitoring may be the preferred treatment option. However, follicular lymphoma may transform into DLBCL, a more aggressive form of the disease.
    • Treatment options for follicular lymphoma include targeted therapy, chemotherapy and radiation therapy.
  • Mantle cell lymphoma (MCL): Accounting for about 5 percent of all lymphoma diagnoses, MCL is more commonly diagnosed in older men. This slow-growing disease is most often found in the lymph nodes, spleen and bone marrow. In most cases, MCL forms when segments of two chromosomes switch places, or translocate. MCL may cause swollen lymph nodes and may spread to the blood or gastrointestinal tract.
    • Treatment options for MCL may include targeted therapy, chemotherapy and stem cell transplants. In some cases, if the cancer is especially slow-growing, surveillance and monitoring may be an option.
  • Marginal zone lymphomas: This slow-growing lymphoma forms in mature B cells circulating in the spleen. Treatment options for marginal zone lymphomas may include chemotherapy and, in some cases, surgery. If the disease is associated with an infection, antibiotics may be prescribed. Marginal zone lymphomas are categorized by three main types, which account for about up to 10 percent of all lymphomas:
    • Mucosa-associated lymphoid tissue (MALT) lymphoma: Also known as extranodal marginal zone B-cell lymphoma, this is the most common type of marginal zone lymphoma. MALT lymphomas often originate in the stomach and are linked to an infection related to stomach ulcers. MALT lymphoma may also form in the lung, skin, thyroid, salivary glands or tissues surrounding the eye. This lymphoma is typically confined to its original location and does not spread. Some MALT lymphomas have been linked to infections with bacteria or viruses.
    • Nodal marginal zone B-cell lymphoma: Also called monocytoid B-cell lymphoma, this rare disease is usually found in the lymph nodes and sometimes in bone marrow. It is more common in older women.
    • Splenic marginal zone B-cell lymphoma: This disease is found in the spleen and bone marrow, and is most common in older men. In some cases, this lymphoma may be linked to infection with the hepatitis C virus.

Less common forms of B-cell lymphoma include:

  • Burkitt lymphoma: Considered the most aggressive form of lymphoma, this disease is one of the fastest growing of all cancers. Burkitt lymphoma, named for the surgeon who first identified the cancer in the 1950s, accounts for about 2 percent of all lymphoma diagnoses.
  • Lymphoplasmacytic lymphoma: This rare cancer, also known as Waldenstrom macroglobulinemia, develops in the bone marrow. Waldenstrom cells produce an overabundance of antibodies called macroglobulins, which may build up in the marrow and crowd out healthy cells. Symptoms include vision problems, headaches and excessive bleeding. The disease is named for Swedish scientist Jan Gosta Waldenstrom.

T-cell lymphomas

T-cell non-Hodgkin lymphoma, which accounts for about 10 percent of all cases, is categorized by various cutaneous T-cell lymphoma types, including mycosis fungoides, and other peripheral T-cell lymphomas.

Natural killer cell lymphoma, or NK cell lymphoma, is diagnosed in less than 1 percent of cases, but it may be extremely aggressive. Natural killer cells are hybrid immune cells that share some characteristics with T cells. This type of lymphoma is most often found in the sinuses and may be referred to as nasal NK/T-cell lymphoma. It may also be found in the windpipe on the gastrointestinal tract.

Some types of T-cell lymphomas include:

Cutaneous T-cell lymphomas: These lymphomas are found in mature T cells, as opposed to immature cells. Types of cutaneous lymphomas include:

  • Mycosis fungoides, also known as Alibert-Bazin syndrome, is a low-grade type of non-Hodgkin lymphoma in which white blood cells become cancerous and affect the skin. Mycosis fungoides are so named because cells in advanced stages of the disease look like mushroom fungus. Mycosis fungoides generally affects the skin, but it may progress to internal organs or the lymph nodes.
  • Sezary syndrome, a more aggressive type of non-Hodgkin lymphoma, is diagnosed when cancerous lymphoma cells that would normally be found on the skin are also found in the blood, lymph nodes and other areas of the body.
  • Primary cutaneous anaplastic large-cell lymphoma is a very rare but aggressive cancer found in mature T cells is most often found in children and young adults, but it is also diagnosed in older patients.
  • Peripheral T-cell lymphomas are often aggressive cancers found in mature T cells. Taken individually, these cancers are rare, but they collectively account for up to 15 percent of all non-Hodgkin lymphoma cases. About 25 percent of peripheral T-cell lymphomas do not fit into one of the categories listed below and are considered “peripheral T-cell lymphoma not otherwise specified.” Some examples of peripheral T-cell lymphomas include:
    • Angioimmunoblastic T-cell lymphoma (AITL), a type of lymphoma, most often diagnosed in older adults, is typically found in the spleen and/or liver and may cause symptoms of autoimmune disease. According to the Lymphoma Research Foundation, most cases of AITL are diagnosed at stage III or stage IV. AITL accounts for about 2 percent to 4 percent of all lymphomas and up to 15 percent of all peripheral lymphomas.
    • Anaplastic large-cell lymphoma (ALCL) accounts for about 2 percent of all non-Hodgkin lymphomas and 15 percent of all peripheral lymphomas. ALCL is most often diagnosed in children and young adults, but it may also affect older adults. Subtypes of ALCL include:
      • Primary cutaneous ALCL, which is found in the skin
      • Systemic ALCL, which may affect lymph nodes and organs
    • Enteropathy-type T-cell lymphoma (EATL), a type of T-cell lymphoma that develops in the intestines, is associated with celiac disease, an autoimmune condition typically caused by a gluten allergy or intolerance. A less-common form of EATL is not linked to celiac disease.
    • Hepatosplenic gamma/delta T-cell lymphoma is a very rare bu aggressive disease that forms in the spleen.

Symptoms of peripheral T-cell lymphoma may include rashes or red skin patches or lesions, itching and swollen lymph nodes. Treatments for peripheral T-cell lymphoma may include chemotherapy, delivered in high doses and/or in combination with stem cell transplants.

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