Lymphoma Non-Hodgkins lymphoma



A lymphoma other than Hodgkin disease, classified by Rappaport into a nodular or diffuse tumor pattern and by cell type; a working or international formulation separates such lymphomas into low, intermediate, and high grade malignancy and into cytologic subtypes reflecting follicular center cell or other origin.

Alternative Names: Lymphoma - non-Hodgkin's; Lymphocytic lymphoma; Histiocytic lymphoma; Lymphoblastic lymphoma; Cancer - non-Hodgkin's lymphoma

Abbreviation: NHL.

The lymphatic system, which is the target of lymphomas, includes the lymph nodes and other organs that make up the immune and blood-forming (hematopoietic) elements of the body.

The lymph nodes are oval, pea-sized organs. They are found beneath the skin along the route of large blood vessels, and are grouped in areas such as the neck, underarms, groin, abdomen (trunk), and pelvis (hips). Lymph nodes are linked throughout the body by narrow tubes called lymphatic vessels. These vessels carry lymph (colorless liquid that is collected from the body's tissues), chyle (milky fluid taken from food in the intestine during digestion), lymphocytes (specialized white blood cells), and other blood cells. The lymphatic fluids and lymphocytes ultimately are funneled back into the bloodstream through a connection in the left upper chest.

Other organs that contain lymphatic tissue and can be affected by lymphoma include the following:

    Spleen ("ductless gland" located on the left side of the body under the lower rib cage that makes lymphocytes and other infection-fighting cells, stores healthy blood cells, and filters the blood).

  • Thymus gland (gland located in front of the heart that produces immature T-cells that, when mature, are involved in immune system responses).

  • Bone marrow (inner region of the bones).

  • Adenoids (lymphatic tissue in the post-nasal area).

  • Tonsils (rounded mass of lymphatic tissue at the back of the throat).

Lymphomas are cancers of the lymphatic system—the body's blood-filtering tissues that help to fight infection and disease. Like other cancers, lymphomas, occur when cells divide too much and too fast. Growth control is lost, and the lymphatic cells may overcrowd, invade, and destroy lymphoid tissues and metastasize (spread) to other organs.

Lymphoma is composed of malignant lymphocytes. Lymphocytes are a normally occurring part of the white blood cell series. The lymphocyte population can be simplistically divided into B-cells, T-cells, and null cells. The job of B-cell lymphocytes is to participate in the immune system by producing antibodies. The job of the T-cell lymphocytes is to direct the participation of B-cells and other cell types in an overall immune response; they are the conductors of the immune system. Lymphomas are the malignant counterpart of these normal cells.

Lymphomas are classified in four stages:

    Stage I: In Stage I (early disease), lymphoma is found in only one lymph node area or in only one area or organ outside the lymph nodes.

  • Stage II: In Stage II (locally advanced disease), lymphoma is found in two or more lymph nodes on the same side of the diaphragm or the lymphoma extends from a single lymph node or single group of lymph nodes into a nearby organ.

  • Stage III: In Stage III (advanced disease), lymphoma is found in lymph node areas on above and below the diaphragm. Lymphoma may have also spread into areas or organs adjacent to lymph nodes, such as the spleen.

  • Stage IV: In Stage IV (widespread disease), the lymphoma has spread (metastasized) via the bloodstream to organs outside the lymph system, such as the bone marrow, brain, skin, or liver.

Non-Hodgkin's lymphoma (NHL) is a heterogenous disease. Each year, there are approximately 66,000 new cases and almost 20,000 deaths from NHL in the United States. Unlike Hodgkin's disease, NHL is comprised of approximately 10 different subtypes (in the Working Formulation) and 20 different disease entities in the Revised European-American Lymphoma Classification (REAL) system.

These subtypes are grouped into 3 biologic states- low grade, intermediate grade, and high grade lymphoma. Therapy is determined by several factors, including the biologic state of the lymphoma, the stage of lymphoma, the presence or absence of symptoms (e.g., weight loss, night sweats, organ dysfunction), and the overall general health of the patient.

A number of factors, including congenital and acquired immunodeficiency states, and infectious, physical, and chemical agents, have been associated with an increased risk for NHL. Infectious agents, such as viral infections (e.g., Epstein barr virus, HIV, human T-cell leukemia virus), and bacterial infections (e.g., heliobacter pylori) may be associated with the development of NHL. Additionally, physical and chemical agents such as pesticides, solvents, arsenate, and lead, as well as hair dyes, radiation exposure (high dose), and paint thinners may also increase the risk.

Non-Hodgkin's lymphoma occurs more often in patients between the ages of 40 and 70. Risk for disease recurrence and overall survival rate can be predicted by using an international prognostic index (IPI) which takes into account age, stage of disease, general health (also known as performance status), number of extra nodal sites, and presence or absence of an elevated serum enzyme named LDH (lactate dehydrogenase).


The signs and symptoms of non-Hodgkin's lymphoma vary from person to person depending on the type of lymphoma and where a tumor is located. Some people may feel stomach pain, constipation, and decreased appetite. Others may have trouble breathing, difficulty swallowing, and notice coughing, wheezing, or chest pain. Other symptoms may include:

    Painless swollen lymph nodes.

  • Fever, chills, or night sweats.

  • Itchy skin.

  • Weight loss despite eating normally.

  • Tiredness.

  • Bone or joint pain.

  • Recurring infections.

The symptom that some people notice first is swollen lymph nodes — usually in the neck, armpits, and groin. Of course, swollen lymph nodes do not usually mean cancer — they're most often a sign of a common illness, like an infection. In fact, all of the symptoms of non-Hodgkin's lymphoma can also be caused by other conditions, which is why only a doctor can determine what's really wrong.

Causes and Risk factors:

An exact cause of Non-Hodgkin Lymphoma is still unknown. Scientists have not yet identified what exactly makes a lymphocyte go haywire and transform into a lymphoma cell. But there are some conditions that make a person more likely to get a Non-Hodgkin Lymphoma are:

A cancer caused by microbes: An association has been found between Non-Hodgkin Lymphoma and certain infections - HTLV-1, Epstein-Barr (EBV) virus, Hepatitis C virus, Helicobacter pylori, a bacteria that may cause lymphomas of the stomach and HIV, the "AIDS" virus. Infection with any of these increases the risk of later development of lymphoma. It is possible that these microbes may enter the lymphocytes and change them into cancer cells.

A weak immune system: Lymphoma is a cancer of the immune system. Conditions which weaken the immune system may also result in a higher risk of developing lymphomas - The most talked about is HIV, which caused AIDS ( a kind of immune failure disease). Some inherited diseases affecting the immune system e.g., ataxia telangiectasia.

Previous treatment for other cancers: Individuals who have received chemotherapy or radiation therapy for previous cancers have a slightly greater chance of developing Non-Hodgkin Lymphoma.Rarely persons suffering from Hodgkin Lymphoma can change to Non-Hodgkin Lymphoma many years after treatment.

Risk Factors:

Age: Non-Hodgkin's lymphoma can develop in people of all ages, including children, it is most common in adults. The most common types of NHL usually appear in people in their 60s and 70s.

Gender: In general, NHL is more common in men than in women.

Race: Overall, the risk for NHL is slightly higher in Caucasians than in African-Americans and Asian Americans.

Family History: People who have close family relatives who have developed NHL may be at increased risk for this cancer. However, no definitive hereditary or genetic link has been established.

Infections: Viral or bacterial infections may play a role in some lymphomas. These include:

    Epstein-Barr virus (EBV), the cause of mononucleosis, is highly associated with Burkitt's disease and NHLs associated with immunodeficiency diseases. It is also a risk factor for Hodgkin's disease.

  • The human immunodeficiency virus (HIV), which causes AIDS, increases the risk for Burkitt lymphoma and diffuse large B-cell lymphoma The hepatitis C virus (HCV) may increase the risk for certain types of lymphomas.

  • The Helicobacter pylori bacterium, which causes stomach ulcers, is associated withincreased risk for mucosa-associated lymphoid tissue lymphomas (MALT). (The use of antibiotics to get rid of the bacteria may cause remission in some patients who have an early stage form of lymphoma in an early stage.)

Autoimmune Disorders: Patients with a history of autoimmune diseases, including rheumatoid arthritis (RA), systemic lupus erythematosus, Hashimoto's thyroiditis, Crohn's disease, and Sj


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