Cancer Types


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Lymphoma (Non-Hodgkin's Lymphoma or NHL)    see video

(Hodgkin's Disease is on another page)

This area of cancer has become quite complex because of the many different types of lymphoma. To keep things simple, lymphomas are separated into indolent (or low grade) and aggressive (or high grade.) Lymphomas typically arise in a lymphatic structure (lymph nodes, spleen or bone marrow) but quite commonly can arise in a non-lymphatic structure (e.g. brain, breast, thyroid, etc, and are called extranodal.) The cellular classification of various histologic subtypes has become quite complex see below and from the NCI  (go here). See the  recent online updates (go here and here) and read the NCCN patient guidelines (go here.) and phsician site here

Subtypes of non-Hodgkin lymphoma
There are many different types of non-Hodgkin lymphoma. The types are generally described by how quickly the cancer is growing: low grade (indolent), and high-grade (aggressive) forms. NHL is more common in adults, in whom the low grade and aggressive NHLs are about equally common. High-grade non-Hodgkin lymphomas are most common in children.

Low grade lymphomas are slow-growing (indolent). These types of lymphoma grow very slowly and tend to be advanced by the time they cause symptoms. Around 85% to 90% of patients with these conditions have advanced (stage IV, see below) disease when they first visit an oncologist. They grow slowly, and usually respond well to several different types of treatment. They are usually not curable.

High grade lymphomas are rapidly growing (aggressive). These types of lymphoma grow rapidly, sometimes very rapidly. These conditions usually require chemotherapy that is sometimes very intensive. These conditions are often curable.

Some lymphomas cannot be easily classified as low grade or high grade. For example, mantle cell lymphoma has features of both high grade and low grade lymphoma.

There are 35 recognized subtypes of non-Hodgkin lymphoma. Doctors use symptoms, blood tests and genetic analyses to determine the subtype. It is very important to identify each person's subtype because they can behave very differently, and require different treatments. They are classified using the World Health Organization Classification.

Subtypes describe the form of the cancer cells and the structure of the cancerous lymphatic tissue when viewed under the microscope. Follicular lymphoma cells form clusters, also known as follicles, while diffuse cells are evenly distributed through lymphatic tissue. Non-Hodgkin lymphomas also are classified by whether they affect the B or T cells of the immune system. Some subtypes are described by the parts of the body involved: the lymph nodes (nodal), spleen (splenic), chest area (mediastinal) or other organs (extranodal).

B-cell lymphomas make up about 90% of all cases of lymphoma. The most common subtypes of B-cell lymphoma:

Diffuse large B-cell lymphoma: The most common form of lymphoma (about 30% of all cases of NHL). Occurs outside the lymph nodes in about 40% of cases, and is considered an aggressive form of NHL. People with diffuse large cell lymphomas most often have large masses, weight loss, night sweats, and fever. This type of lymphoma is very often curable with a combination of radiation therapy and doxorubicin-based chemotherapy. Some people may also receive chemotherapy to the central nervous system to prevent the lymphoma from spreading (called CNS prophylaxis).

Follicular lymphoma: The second most common form of lymphoma in the United States and Europe, about 20% of all cases of NHL. It usually begins in the lymph nodes, is most often low grade, and moves very slowly. There is no known cure, and most people survive 8 to 10 years after diagnosis. People with follicular lymphomas may be treated with combination chemotherapy, or may be followed closely with watchful waiting. Stem cell transplants, tumor vaccines, interferon, and monoclonal antibody treatments may also be available in clinical trials.

Splenic marginal zone B-cell lymphoma (with or without villous lymphocytes): a form of lymphoma that begins in the spleen. It is an uncommon form of B-cell chronic lymphocytic lymphoma (CLL). It is usually slow-acting and often requires no treatment. Sometimes, surgical removal of the spleen is recommended.

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type: most commonly occurs in the stomach; also in the lung, thyroid, salivary gland, or eye. People with this form of lymphoma often have a history of autoimmune disease. When MALT occurs in the stomach, it is often treated successfully with antibiotics. In other cases, radiation, surgery, chemotherapy, or a combination of these is the most common treatment. In general, these lymphomas are treated similarly to diffuse large cell lymphoma.

Nodal marginal zone B-cell lymphoma (with or without monocytoid B cells). Involves the lymph nodes. Rare, about 1% of all lymphomas. In general, these lymphomas are treated similarly to diffuse large cell lymphoma.

Lymphoplasmacytic lymphoma: Rare (1% of all NHL), indolent (low grade) form of lymphoma. This form of lymphoma most often involves the bone marrow, lymph nodes, and spleen. People with lymphoplasmacytic lymphoma often experience hyperviscosity (thickened blood) that may cause them to feel chills. Treatments are similar to chronic lymphocytic lymphoma/leukemia, and may include combination chemotherapy, watchful waiting, interferon, or monoclonal antibodies. Chemotherapy followed by stem cell transplant is being explored in clinical trials.

Mantle cell lymphoma: An aggressive form of NHL that makes up 7% of all NHL. It most often appears with no symptoms in people over age 60. It most often involves the bone marrow, lymph nodes, spleen, and gastrointestinal system, and is identified by its expression of a protein called the cyclin D1 protein. People with mantle cell lymphoma also exhibit a translocation of two chromosomes (11 and 14), which means that parts of these two chromosomes have traded places. Mantle cell lymphoma is considered an aggressive type of lymphoma, and often does not respond or stops responding to chemotherapy. Clinical trials of mantle cell lymphoma using high dose chemotherapy followed by stem cell transplant, interferon, or monoclonal antibodies after CHOP chemotherapy are in progress.

Mediastinal large B-cell lymphoma: An aggressive form of diffuse large B-cell lymphoma that appears as a large mass in the chest area that may cause respiratory symptoms or superior vena cava (SVC) syndrome. It is most common in women between the ages of 30 and 40, and represents about 2.5% of all NHL cases. It is treated with high doses of chemotherapy, and may also respond to stem cell transplant.

Primary effusion lymphoma: This very aggressive form of lymphoma most often occurs in people who are HIV-positive. It appears in the lung, heart, or abdominal cavities, and often there is not one identifiable tumor mass. It is treated as other diffuse large cell lymphomas.

Burkitt's lymphoma/Burkitt's cell leukemia: A very rare and aggressive form of lymphoma. There are three forms of Burkitt's lymphoma: endemic, sporadic, and immunodeficiency-related. It occurs most commonly in Africa, in the jawbones of children, and is associated with the Epstein-Barr virus. Burkitt's lymphoma is identified by a specific translocation and inactivity of the c-myc gene on chromosomes. It is an aggressive type of lymphoma, and is often curable with doxorubicin-based chemotherapy. Bone marrow transplants for Burkitt's lymphoma are underway.

T-cell lymphomas make up about 10% of all lymphoma cases. The most common subtypes of T- and natural killer (NK-) cell lymphomas:

Precursor T-lymphoblastic lymphoma/leukemia (precursor T-cell acute lymphoblastic leukemia): Makes up about 15% of all childhood and about 25% of all adult ALL (acute lymphocytic lymphoma)[link]. It is most common in late childhood, adolescence, and early adulthood, and is more common in men. It is called leukemia if there are more than 25% bone marrow blasts (early white blood cells); and lymphoma is there are fewer than 25% bone marrow blasts and a mass forming in the chest area (mediastinal mass). Lymphoblastic lymphoma most often begins as a large mass in the chest area that moves quickly to the bone marrow. This is considered a very aggressive form of lymphoma. It is treated with intensive chemotherapy with bone marrow transplant, and may include radiation therapy.

Adult T-cell lymphoma/leukemia (human T-cell lymphotropic virus type I positive): This type of lymphoma is caused by a virus called the human T-cell lymphotropic virus type I. It is an aggressive disease that most often involves the bone and skin; often leukemia cells are found in the blood. This form of lymphoma usually has a poor response to chemotherapy, though some successes have been seen with zidovudine and interferon. About two thirds of patients experience remission.

Extranodal NK/T-cell lymphoma, nasal type: This is an aggressive type of lymphoma, very rare in the United States and Europe but more common in Asian and Hispanic communities. It can occur in children or adults, and most often involves the nasal area, but can also involve the trachea, gastrointestinal tract, or skin. It often does not respond well to standard chemotherapy, but may be treated with radiation therapy followed by chemotherapy. Bone marrow or peripheral stem cell transplantation for this type of lymphoma is being studied in clinical trials.

Enteropathy type T-cell lymphoma: Rare in the United States and Europe. This is an aggressive form of T-cell lymphoma that involves the intestines of people who have celiac disease (gluten intolerance). If left untreated, it can damage the intestines severely. This type of lymphoma is preventable if people who know they have celiac disease adopt a gluten-free diet. High dose chemotherapy may be used to treat enteropathy type T-cell lymphoma.

Hepatosplenic T-cell lymphoma: This is an aggressive form of peripheral T-cell lymphoma that involves the liver and spleen. It occurs most often in male adolescents and young men. It is treated as a high-risk diffuse large cell lymphoma.

Subcutaneous panniculitis-like T-cell lymphoma: This is a form of peripheral T-cell lymphoma that involves the tissues under the skin, and is often first diagnosed as panniculitis (inflammation of fatty tissues). It is treated as a high-risk diffuse large cell lymphoma.

Anaplastic large cell lymphoma, T/null-cell, primary cutaneous type: This is an aggressive form of lymphoma that involves the skin only. It is often successfully treated with doxorubicin-based chemotherapy.

Peripheral T-cell lymphoma: This is an aggressive form of lymphoma, most often discovered at an advanced stage. It is most common people over age 60, and makes up about 6% of all lymphomas in the United States and Europe. It involves either diffuse large or diffuse mixed cells that express certain types of proteins (CD4 or CD8) on a blood test. It is treated like diffuse large B-cell lymphoma, with doxorubicin-based chemotherapy. Stem cell transplantation may be considered in some cases.

Angioimmunoblastic T-cell lymphoma: This is a moderately aggressive form of lymphoma marked by the symptoms it produces: swollen lymph nodes, fever, weight loss, skin rash, and high levels of antibodies called gamma globulin in the blood. Since people with angioblastic lymphoma have depressed immune systems, infections are also common. This type of lymphoma is identified by specific genetic alterations on the T-cell receptors. It is managed like other diffuse large cell lymphomas.

Anaplastic large cell lymphoma, T/null cell, primary systemic type: This form makes up about 2% of all lymphomas, and about 10% of all childhood lymphomas. It occurs in both adults and children. It is an aggressive form of lymphoma but often responds well to treatment.