Non-Hodgkin's lymphoma
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Non-Hodgkin's lymphoma is a type of cancer. Lymphoma is a general term for cancers that develop in the lymphatic system. Hodgkin's disease is one type of lymphoma. All other lymphomas are grouped together and are called non-Hodgkin's lymphoma. Lymphomas account for about 5 percent of all cases of cancer in the United States.
The lymphatic system is part of the body's immune system. It helps the body fight disease and infection. The lymphatic system includes a network of thin tubes that branch, like blood vessels, into tissues throughout the body. Lymphatic vessels carry lymph, a colorless, watery fluid that contains infection-fighting cells called lymphocytes. Along this network of vessels are small organs called lymph nodes. Clusters of lymph nodes are found in the underarms, groin, neck, chest, and abdomen. Other parts of the lymphatic system are the spleen, thymus, tonsils, and bone marrow. Lymphatic tissue is also found in other parts of the body, including the stomach, intestines, and skin.
Cancer is a group of many related diseases that begin in cells, the body's basic unit of life. To understand non-Hodgkin's lymphoma, it is helpful to know about normal cells and what happens when they become cancerous. The body is made up of many types of cells. Normally, cells grow and divide to produce more cells only when the body needs them. This orderly process helps keep the body healthy. Sometimes cells keep dividing when new cells are not needed, creating a mass of extra tissue. This mass is called a growth or tumor. Tumors can be either benign (not cancerous) or malignant (cancerous).
In non-Hodgkin's lymphoma, cells in the lymphatic system become abnormal. They divide and grow without any order or control, or old cells do not die as cells normally do. Because lymphatic tissue is present in many parts of the body, non-Hodgkin's lymphoma can start almost anywhere in the body. Non-Hodgkin's lymphoma may occur in a single lymph node, a group of lymph nodes, or in another organ. This type of cancer can spread to almost any part of the body, including the liver, bone marrow, and spleen.
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Symptoms
The most common symptom of non-Hodgkin's lymphoma is a painless swelling of the lymph nodes in the neck, underarm, or groin.
Other symptoms may include the following:
- Unexplained fever
- Night sweats
- Constant fatigue
- Unexplained weight loss
- Itchy skin
- Reddened patches on the skin
When symptoms like these occur, they are not sure signs of non-Hodgkin's lymphoma. They may also be caused by other, less serious conditions, such as the flu or other infections. Only a doctor can make a diagnosis. When symptoms are present, it is important to see a doctor so that any illness can be diagnosed and treated as early as possible. Do not wait to feel pain; early non-Hodgkin's lymphoma may not cause pain.
Diagnosis
If non-Hodgkin's lymphoma is suspected, the doctor asks about the person's medical history and performs a physical exam. The exam includes feeling to see if the lymph nodes in the neck, underarm, or groin are enlarged. In addition to checking general signs of health, the doctor may perform blood tests.
The doctor may also order tests that produce pictures of the inside of the body. These may include:
- X-rays: Pictures of areas inside the body created by high-energy radiation.
- CT scan (also known as a "CAT scan"): A series of detailed pictures of areas inside the body. The pictures are created by a computer linked to an x-ray machine.
- MRI (magnetic resonance imaging): Detailed pictures of areas inside the body produced with a powerful magnet linked to a computer.
- Lymphangiogram: Pictures of the lymphatic system taken with x-rays after a special dye is injected to outline the lymph nodes and vessels.
A biopsy is needed to make a diagnosis. A surgeon removes a sample of tissue so that a pathologist can examine it under a microscope to check for cancer cells. A biopsy for non-Hodgkin's lymphoma is usually taken from a lymph node, but other tissues may be sampled as well. Sometimes, an operation called a laparotomy may be performed. During this operation, a surgeon cuts into the abdomen and removes samples of tissue to be checked under a microscope.
A patient who needs a biopsy may want to ask the doctor some of the following questions:
- Why do I need to have a biopsy?
- How long will the biopsy take? Will it hurt?
- How soon will I know the results?
- If I do have cancer, who will talk with me about treatment? When?
Types of Non-Hodgkin's Lymphoma
Over the years, doctors have used a variety of terms to classify the many different types of non-Hodgkin's lymphoma. Most often, they are grouped by how the cancer cells look under a microscope and how quickly they are likely to grow and spread. Aggressive lymphomas, also known as intermediate and high-grade lymphomas, tend to grow and spread quickly and cause severe symptoms. Indolent lymphomas, also referred to as low-grade lymphomas, tend to grow quite slowly and cause fewer symptoms. One of the paradoxes of non-Hodgkin's lymphoma is that the indolent lymphomas generally cannot be cured by chemotherapy, while in a significant number of cases aggressive lymphomas can be. Current lymphoma classification is complex. Common types of lymphomas include follicular lymphoma and diffuse large B cell lymphoma. Two relatively uncommon lymphomas are caused by viruses: Burkitt's lymphoma is caused by the Epstein-Barr virus, while adult T-cell lymphoma/leukemia is caused by the HTLV-1 virus, a virus related to the one that causes AIDS; the former is endemic in Africa and the latter in parts of Japan and the Caribbean. However, the etiology of most lymphomas is not known.
Staging
If non-Hodgkin's lymphoma is diagnosed, the doctor needs to learn the stage, or extent, of the disease. Staging is a careful attempt to find out whether the cancer has spread and, if so, what parts of the body are affected. Treatment decisions depend on these findings.
The doctor considers the following to determine the stage of non-Hodgkin's lymphoma:
- The number and location of affected lymph nodes;
- Whether the affected lymph nodes are above, below, or on both sides of the diaphragm (the thin muscle under the lungs and heart that separates the chest from the abdomen); and
- Whether the disease has spread to the bone marrow, spleen, or to organs outside the lymphatic system, such as the liver.
In staging, the doctor may use some of the same tests used for the diagnosis of non-Hodgkin's lymphoma. Other staging procedures may include additional biopsies of lymph nodes, the liver, bone marrow, or other tissue. A bone marrow biopsy involves removing a sample of bone marrow through a needle inserted into the hip or another large bone. A pathologist examines the sample under a microscope to check for cancer cells.
Stages of NHL
The various stages of NHL (the Ann Arbor staging classification, developed for Hodgkin's lymphoma) are based on how far the cancer has spread throughout and beyond the lymphatic system, and whether constitutional symptoms (fever, night sweats, or weight loss) are present.
- Stage I
- "Stage I" indicates that the cancer is located in a single region, usually one lymph node and the surrounding area. Stage I often will not have outward symptoms.
- Stage II
- "Stage II" indicates that the cancer is located in two separate regions, an affected lymph node or organ within the lymphatic system and a second affected area, and that both affected areas are confined to one side of the diaphagm - that is, both are above the diaphragm, or both are below the diaphragm.
- Stage III
- "Stage III" indicates that the cancer has spread to both sides of the diaphragm, including one organ or area near the lymph nodes or the spleen.
- Stage IV
- "Stage IV" indicates that the cancer has spread beyond the lymphatic system and involves one or more major organs, possibly including the bone marrow or skin.
The absence of constitutional symptoms is denoted by adding an "A" to the stage; the presence is denoted by adding a "B" to the stage.
Staging in Non-Hodkin's lymphomas is far less significant in determining therapy than it is in Hodgkin's lymphoma.
Treatment
The doctor develops a treatment plan to fit each patient's needs. Treatment for non-Hodgkin's lymphoma depends on the stage of the disease, the type of cells involved, whether they are indolent or aggressive, and the age and general health of the patient.
Non-Hodgkin's lymphoma is often treated by a team of specialists that may include a hematologist, medical oncologist, and/or radiation oncologist. Non-Hodgkin's lymphoma is usually treated with chemotherapy, radiation therapy, or a combination of these treatments. In some cases, bone marrow transplantation, biological therapies, or surgery may be options. For indolent lymphomas, the doctor may decide to wait until the disease causes symptoms before starting treatment. Often, this approach is called "watchful waiting."
Taking part in a clinical trial (research study) to evaluate promising new ways to treat non-Hodgkin's lymphoma is an important option for many people with this disease.
Getting a Second Opinion
Before starting treatment, patients may want a second opinion to confirm their diagnosis and treatment plan. Some insurance companies require a second opinion; others may cover a second opinion if the patient or doctor requests it.
There are a number of ways to find a doctor who can give a second opinion:
- The patient's doctor may be able to suggest specialists to consult.
- In the United States, The Cancer Information Service, at 1-800-4-CANCER, can tell callers about cancer treatment facilities, including cancer centers and other programs supported by the National Cancer Institute.
- Patients can get the names of doctors from their local medical society, a nearby hospital, or a medical school.
- In the United States, The Official ABMS Directory of Board Certified Medical Specialists lists doctors' names along with their specialty and medical background. This resource, produced by the American Board of Medical Specialties, is available in most public libraries and on the Internet.
Preparing for Treatment
Many people with cancer want to learn all they can about their disease and their treatment choices so they can take an active part in decisions about their medical care. When a person is diagnosed with cancer, shock and stress are natural reactions. These feelings may make it difficult for people to think of everything they want to ask the doctor. Often, it helps to make a list of questions. To help remember what the doctor says, patients may take notes or ask whether they may use a tape recorder. Some people also want to have a family member or friend with them when they talk to the doctor -- to take part in the discussion, to take notes, or just to listen.
These are some questions a patient may want to ask the doctor before treatment begins:
- What kind of non-Hodgkin's lymphoma do I have?
- What is the stage of the disease?
- What are my treatment choices? Which do you recommend for me? Why?
- What are the risks and possible side effects of each treatment?
- What side effects should I report to you?
- How long will treatment last?
- What are the chances that the treatment will be successful?
- Will treatment affect my normal activities? If so, for how long?
- Are new treatments under study? Would a clinical trial be appropriate for me?
- What is the treatment likely to cost?
Patients do not need to ask all their questions or remember all the answers at one time. They will have other chances to ask the doctor to explain things and to get more information.
Methods of Treatment
Chemotherapy and radiation therapy are the most common treatments for non-Hodgkin's lymphoma, although bone marrow transplantation, biological therapies, or surgery are sometimes used.
Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy for non-Hodgkin's lymphoma usually consists of a combination of several drugs. Patients may receive chemotherapy alone or in combination with radiation therapy.
Chemotherapy is usually given in cycles: a treatment period followed by a recovery period, then another treatment period, and so on. Most anticancer drugs are given by injection into a blood vessel (IV); some are given by mouth. Chemotherapy is a systemic treatment because the drugs enter the bloodstream and travel throughout the body.
Usually a patient has chemotherapy as an outpatient (at the hospital, at the doctor's office, or at home). However, depending on which drugs are given and the patient's general health, a short hospital stay may be needed.
Radiation therapy (also called radiotherapy) is the use of high-energy rays to kill cancer cells. Treatment with radiation may be given alone or with chemotherapy. Radiation therapy is local treatment; it affects cancer cells only in the treated area. Radiation therapy for non-Hodgkin's lymphoma comes from a machine that aims the high-energy rays at a specific area of the body. There is no radioactivity in the body when the treatment is over.
Sometimes patients are given chemotherapy and/or radiation therapy to kill undetected cancer cells that may be present in the central nervous system (CNS). In this treatment, called central nervous system prophylaxis, the doctor injects anticancer drugs directly into the cerebrospinal fluid.
Bone marrow transplantation (BMT) may also be a treatment option, especially for patients whose non-Hodgkin's lymphoma has recurred (come back). BMT provides the patient with healthy stem cells (very immature cells that produce blood cells) to replace cells damaged or destroyed by treatment with very high doses of chemotherapy and/or radiation therapy. The healthy bone marrow may come from a donor, or it may be marrow that was removed from the patient, treated to destroy cancer cells, stored, and then given back to the person following the high-dose treatment. Until the transplanted bone marrow begins to produce enough white blood cells, patients have to be carefully protected from infection. They usually stay in the hospital for several weeks.
Biological therapy (also called immunotherapy) is a form of treatment that uses the body's immune system, either directly or indirectly, to fight cancer or to lessen the side effects that can be caused by some cancer treatments. It uses materials made by the body or made in a laboratory to boost, direct, or restore the body's natural defenses against disease. Biological therapy is sometimes also called biological response modifier therapy.
Clinical Trials
Many people with non-Hodgkin's lymphoma take part in clinical trials (research studies). Doctors conduct clinical trials to learn about the effectiveness and side effects of new treatments. In some trials, all patients receive the new treatment. In others, doctors compare different therapies by giving the new treatment to one group of patients and the standard therapy to another group; or they may compare one standard treatment with another. Research like this has led to significant advances in the treatment of cancer. Each achievement brings researchers closer to the eventual control of cancer.
Doctors are studying radiation therapy, new ways of giving chemotherapy, new anticancer drugs and drug combinations, biological therapies, bone marrow transplantation, peripheral blood stem cell transplantation, and new ways of combining various types of treatment. Some studies are designed to find ways to reduce the side effects of treatment and to improve the patient's quality of life.
Side Effects of Treatment
Treatments for non-Hodgkin's lymphoma are very powerful. It is hard to limit the effects of therapy so that only cancer cells are removed or destroyed. Because treatment also damages healthy cells and tissues, it often causes side effects.
The side effects of cancer treatment depend mainly on the type and extent of the therapy. Side effects may not be the same for everyone, and they may even change from one treatment to the next. Doctors and nurses can explain the possible side effects of treatment. They can also lessen or control many of the side effects that may occur during and after treatment.
Chemotherapy
The side effects of chemotherapy depend mainly on the drugs and the doses the patient receives. As with other types of treatment, side effects may vary from person to person.
Anticancer drugs generally affect cells that divide rapidly. In addition to cancer cells, these include blood cells, which fight infection, help the blood to clot, or carry oxygen to all parts of the body. When blood cells are affected, the patient is more likely to get infections, may bruise or bleed easily, and may feel unusually weak and tired. The patient's blood count is monitored during chemotherapy and, if necessary, the doctor may decide to postpone treatment to allow blood counts to recover.
Cells in hair roots also divide rapidly; therefore, chemotherapy may lead to hair loss. Patients may have other side effects such as poor appetite, nausea and vomiting, or mouth and lip sores. They may also experience dizziness and darkening of skin and fingernails. Another common side effect is peripheral neuropathy.
Most side effects go away gradually during the recovery periods between treatments or after treatment is over. However, certain anticancer drugs can increase the risk of developing a second cancer later in life.
In some men and women, chemotherapy causes a loss of fertility (the ability to produce children). Loss of fertility may be temporary or permanent, depending on the drugs used and the patient's age. For men, sperm banking before treatment may be an option. Women's menstrual periods may stop, and they may have hot flashes and vaginal dryness. Menstrual periods are more likely to return in young women. The National Cancer Institute booklet Chemotherapy and You has helpful information about chemotherapy and coping with side effects.
Radiation Therapy
The side effects of radiation depend on the treatment dose and the part of the body that is treated. During radiation therapy, people are likely to become extremely tired, especially in the later weeks of treatment. Rest is important, but doctors usually advise patients to try to stay as active as they can.
It is common to lose hair in the treated area and for the skin to become red, dry, tender, or itchy. There may also be permanent darkening or "bronzing" of the skin in the treated area.
When the chest and neck are treated, patients may have a dry, sore throat and trouble swallowing. Some patients may have tingling or numbness in their arms, legs, and lower back. Radiation therapy to the abdomen may cause nausea, vomiting, diarrhea, or urinary discomfort. Often, changes in diet or medicine can ease these problems.
Radiation therapy also may cause a decrease in the number of white blood cells, cells that help protect the body against infection. If that happens, patients need to be careful to avoid possible sources of infection. The doctor monitors a patient's blood count during radiation therapy. In some cases, treatment may have to be postponed to allow blood counts to recover.
Although the side effects of radiation therapy can be difficult, they can usually be treated or controlled. It may also help to know that, in most cases, side effects are not permanent. However, patients may want to discuss with their doctor the possible long-term effects of radiation treatment on fertility and the increased chance of second cancers after treatment is over. The National Cancer Institute booklet "Radiation Therapy and You" has helpful information about radiation therapy and managing its side effects.
Bone Marrow Transplantation
Patients who have a bone marrow transplant face an increased risk of infection, bleeding, and other side effects from the large doses of chemotherapy and radiation they receive. In addition, graft-versus-host disease (GVHD) may occur in patients who receive bone marrow from a donor. In GVHD, the donated marrow attacks the patient's tissues (most often the liver, the skin, and the digestive tract). GVHD can range from mild to very severe. It can occur any time after the transplant (even years later). In most cases, GVHD can be treated effectively with the use of steriods and other immunosuppressant drugs.
Biological Therapy
The side effects caused by biological therapy vary with the specific type of treatment. These treatments may cause flu-like symptoms such as chills, fever, muscle aches, weakness, loss of appetite, nausea, vomiting, and diarrhea. Patients also may bleed or bruise easily, get a skin rash, or retain fluid. These problems can be severe, but they usually go away after treatment stops.
Surgery
The side effects of surgery depend on the location of the tumor, the type of operation, the patient's general health, and other factors. Although patients are often uncomfortable during the first few days after surgery, the pain can usually be controlled with medicine. People can talk with their doctor or nurse about pain relief. It is also common for patients to feel tired or weak for a while. The length of time it takes to recover from an operation varies for each patient.
Nutrition During Cancer Treatment
Eating well during cancer treatment means getting enough calories and protein to help prevent weight loss and regain strength. Good nutrition often helps people feel better and have more energy.
Some people with cancer find it hard to eat a balanced diet because they may lose their appetite. In addition, common side effects of treatment, such as nausea, vomiting, or mouth sores, can make eating difficult. Often, foods taste different. Also, people being treated for cancer may not feel like eating when they are uncomfortable or tired.
Doctors, nurses, and dietitians can offer advice on how to get enough calories and protein during cancer treatment. Patients and their families also may want to read the National Cancer Institute(USA) booklet Eating Hints for Cancer Patients, which contains many useful suggestions.
Recovery and Outlook
It is natural for anyone facing cancer to be concerned about what the future holds. Understanding the nature of cancer and what to expect can help patients and their loved ones plan treatment, anticipate lifestyle changes, and make quality of life and financial decisions.
Cancer patients frequently ask their doctors or search on their own for an answer to the question, "What is my prognosis?" Prognosis is a prediction of the future course and outcome of a disease and an indication of the likelihood of recovery. However, it is only an estimate. When doctors discuss a patient's prognosis, they are attempting to project what is likely to occur for that individual patient.
Sometimes patients use statistics to try to figure out their chances of being cured. However, statistics reflect the experience of a large group of patients and cannot be used to predict what will happen to a particular patient because no two patients are alike. The prognosis for a person with non-Hodgkin's lymphoma can be affected by many factors, particularly the type and stage of the cancer and the patient's age, general health, and response to treatment. The doctor who is most familiar with a patient's situation is in the best position to help interpret statistics and discuss that person's prognosis.
When doctors talk about surviving cancer, they may use the term remission rather than cure. Although many people with non-Hodgkin's lymphoma are successfully treated, doctors use the term remission because cancer can return. It is important to discuss the possibility of recurrence with the doctor.
Followup Care
People who have had non-Hodgkin's lymphoma should have regular followup examinations after their treatment is over. Followup care is an important part of the overall treatment plan, and people should not hesitate to discuss it with their health care provider. Regular followup care ensures that patients are carefully monitored, any changes in health are discussed, and new or recurrent cancer can be detected and treated as soon as possible. Between followup appointments, people who have had non-Hodgkin's lymphoma should report any health problems as soon as they appear.
Risk Factors Associated with Non-Hodgkin's Lymphoma
The incidence of non-Hodgkin's lymphoma has increased dramatically over the last couple of decades. This disease has gone from being relatively rare to being the fifth most common cancer in the United States. At this time, little is known about the reasons for this increase or about exactly what causes non-Hodgkin's lymphoma.
Doctors can seldom explain why one person gets non-Hodgkin's lymphoma and another does not. It is clear, however, that cancer is not caused by an injury, and is not contagious; no one can "catch" non-Hodgkin's lymphoma from another person.
By studying patterns of cancer in the population, researchers have found certain risk factors that are more common in people who get non-Hodgkin's lymphoma than in those who do not. However, most people with these risk factors do not get non-Hodgkin's lymphoma, and many who do get this disease have none of the known risk factors.
The following are some of the risk factors associated with this disease:
- Age/Sex -- The likelihood of getting non-Hodgkin's lymphoma increases with age and is more common in men than in women.
- Weakened Immune System (AIDS-related lymphoma) -- Non-Hodgkin's lymphoma is more common among people with inherited immune deficiencies, autoimmune diseases, or HIV/AIDS, and among people taking immunosuppressant drugs following organ transplants. (see Post-transplant lymphoproliferative disorder)
- Viruses -- Human T-lymphotropic virus type I (HTLV-1) and Epstein-Barr virus are two infectious agents that increase the chance of developing non-Hodgkin's lymphoma.
- Environment -- People who work extensively with or are otherwise exposed to certain chemicals, such as pesticides, solvents, or fertilizers, have a greater chance of developing non-Hodgkin's lymphoma.
People who are concerned about non-Hodgkin's lymphoma should talk with their doctor about the disease, the symptoms to watch for, and an appropriate schedule for checkups. The doctor's advice will be based on the person's age, medical history, and other factors.
Notable NHL patients
Notable persons treated for Non-Hodgkin's lymphoma include:
- Jackie Kennedy Onassis, widow of former U.S. president John F. Kennedy
- U.S. Senator Paul Tsongas
- British soap opera star Anne Kirkbride
- Velvet Underground guitar player Sterling Morrison
- The former Shah Mohammed Reza Pahlevi of Iran (although this was probably CLL).
- King Hussein of Jordan
- U.S. Nobel Prize laureate Jack S. Kilby, inventor of the integrated circuit
References:
- The original version of this article was edited down from the source above. As a work of an agency of the U.S. Federal Government, this document is a public domain resource that can be used as source material for Wikipedia.
- Please see the NIH Policies page (http://www.cancer.gov/policies/page6) for more information
- The original version of this article was edited down from the source above. As a work of an agency of the U.S. Federal Government, this document is a public domain resource that can be used as source material for Wikipedia.
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