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LYMPHOMA

Arm and Leg Swelling After Lymphoma

* I am a thirteen year survivor of lymphoma, first diagnosed in 1995 - Pat O'Connor 

Arm and Leg Swelling After Lymphoma

With the advent of better and more effective cancer treatments, the survival rate for all cancers has risen dramatically.  With this progress, a new and often misunderstood and misdiagnosed complication has arisen.

Many cancer survivors , having overcome cancer, find themselves with sudden and often unexplained swelling, usually of the arms or of the legs.

This swelling occurs because of one of several factors.

First, the swelling begins after lymph nodes have been removed for cancer biopsies. 

Second, the swelling may start as a result of radiation damage to either the lymph nodes and/or the lymph system.

Due to either the removal of lymph nodes or damage to the lymph system, your body is no longer able to rid itself of excess fluids.  The fluids collect in the limbs effected and swelling beings.

This swelling is called lymphedema. The swelling that occurs is permanent, and while it is not curable it is treatable.

Permanent Leg or Arm Swelling

****In the situation of any permanent leg swelling whether the cause is known or unknown, the diagnoses of lymphedema must be considered****

There are several groups of people who experience leg or arm swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.

Group One

This group includes those who have had the injuries, infections, insect bites, trauma to the leg, surgeries or reaction to a medication. When this swelling does not go away, and becomes permanent it is called secondary lymphedema.

Group Two

Another extremely large group that experiences permanent leg or arm swelling are cancer patients, people who are morbidly obese, or those with the condition called lepedema.  What causes the swelling to remain permanent is that the lymph system has been so damaged that it can no longer operate normally in removing the body's waste fluid.

In cancer patients this  is the result of either removal of the lymph nodes for cancer biopsy, radiation damage to the lymph system, or damage from tumor/cancer surgeries.

This is also referred to as secondary lymphedema.

Group Three

Group three consists of people who have leg or arm  swelling from seemingly unknown reasons.  There may be no injury, no cancer, no trauma, but for some reason the leg simply is swollen all the time.

The swelling may start at birth, it may begin at puberty, or may begin in the 3rd, 4th or even 5th decade of life or sometimes later.

This type of leg or arm  swelling is called primary lymphedema.  It can be caused by a genetic defect, malformation or damage to the lymph system while in the womb or at birth or be part of another birth condition that also effects the lymph system.

This is an extremely serious medical condition that must be diagnosed early, and treated quickly so as to avoid painful, debilitating and even life threatening complications.  Treatment should NOT include the use of diuretics.

What is Lymphedema?

Lymphedema is defined simply as an accumulation of excessive protein rich fluid in the tissues of the leg.  The accumulation of fluid causes the permanent swelling caused by a defective lymph system.

A conservative estimate is that there may be 1-2 million people in the United States with some form of primary lymphedema and two to three million with secondary lymphedema.

What are the symptoms of Lymphedema?

If you are an at risk person for arm lymphedema there are early warning signs you should be aware of.  If you experience any or several of these symptoms, you should immediately make your physician aware of them.

1.)  Unexplained aching, hurting or pain in the arm

2.)  Experiencing "fleeting lymphedema."  This is where the limb may swell, even slightly, then return to normal.  This may be a precursor to full blown arm lymphedema.

3.)  Localized swelling of any area.  Sometimes lymphedema may start as swelling in one area, for example the hand, or between the elbow and hand.  This is an indication of early lymphatic malfunction.

4.)  Any arm inflammation, redness or infection.

5.)  You may experience a feeling of tightness, heaviness or weakness of the arm.

How is Lymphedema Treated?

The preferred treatment today is decongestive therapy. The forms of therapy are complete decongestive therapy (CDT) or manual decongestive therapy (MDT), there are variances, but most involve these two type of treatment.

It is a form of massage therapy where the leg is very gently massaged to actually move the fluid out of the leg and into an area where the lymph system still functions normally.

With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down and/or is taught to use compression wraps to maintain the leg size.

What are some of the complications of lymphedema?

1. Infections such as cellulitis, lymphangitis, erysipelas. This is due not only to the large accumulation of fluid, but it is well documented that lymphodemous limbs are localized immuno-deficient.

2. Draining wounds that leak lymphorrea which is very caustic to surrounding skin tissue and acts as a port of entry for infections.

3. Increased pain as a result of the compression of nerves usually caused by the development of fibrosis and increased build up of fluids.

4. Loss of Function due to the swelling and limb changes.

5. Depression - Psychological coping as a result of the disfigurement and debilitating effect of lymphedema.

6. Deep venous thrombosis again as a result of the pressure of the swelling and fibrosis against the vascular system. Also, can happen as a result of cellulitis, lymphangitis and infections.

7. Sepsis, Gangrene are possibilities as a result of the infections.

8. Possible amputation of the limb.

9. Pleural effusions may result if the lymphatics in the abdomen or chest are to overwhelmed to clear the lung cavity of fluids.

10. Skin complications such as splitting, plaques, susceptibility to fungus and bacterial infections.

11. Chronic localized inflammations.

Can lymphedema be cured?

No, at the present time there is no cure for lymphedema. But it can be treated and managed and most of the complications can be avoided.  Life with lymphedema can still be active and full, with proper treatment, patient education, and patient life style adaptation.

For extensive information on lymphedema, please visit our home page:

Lymphedema People

http://www.lymphedemapeople.com

(c) Copyright 2005 by Pat O'Connor and Lymphedema People. Use of this information for educational purpose is encouraged and permitted.  It  must be available free and without charge and not used for financial renumeration or gain.  Please include an acknowledgement to the author and a link to Lymphedema People.

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Lymphedema

This patient summary is adapted from the summary on lymphedema written by cancer experts for health professionals. This and other credible information about cancer treatment, screening, prevention, supportive care, and ongoing clinical trials, is available from the National Cancer Institute. Lymphedema is a swelling of a part of the body, usually an arm or leg, that is caused by the buildup of lymph. Lymph is a nearly colorless fluid that travels throughout the lymphatic system and carries cells that help fight infection and disease. Lymphedema can be caused by cancer or the treatment of cancer. This brief summary describes lymphedema, its causes and treatment.

OVERVIEW

Lymphedema is the buildup of lymph in the tissues, mainly in the fat just under the skin, and is caused by a problem in the lymphatic system. Lymphedema is an abnormal collection of too much tissue protein, fluid (edema), chronic inflammation, and thickening and scarring of connective tissue.

The lymphatic system is made up of vessels that are similar to blood vessels, but it carries lymph instead of blood. Unlike the blood system, which circulates through every part of the body, the lymphatic system drains lymph away from each part of the body. The lymphatic system collects fluid and protein from body tissues and returns it to the blood stream. Proteins and substances too big to fit into veins are moved through the lymphatic system. Edema may occur when these substances are in excess and the lymphatic system becomes overwhelmed or blocked.

Lymphedema is categorized as either primary or secondary. Primary lymphedema is a rare inherited condition in which lymph nodes and lymph vessels are absent or abnormal. Secondary lymphedema is usually caused when the lymphatic system is blocked or cut and may be caused by infection, cancer, or scar tissue. The most frequently affected lymph nodes are in the pelvis and legs or under the arms.

Acute versus chronic lymphedema There are several types of lymphedema. The first type is acute, temporary, and mild lymphedema that occurs within a few days after surgery that has involved the cutting of lymphatic vessels. It usually gets better within a week after keeping the affected arm or leg raised and by pumping the muscle that is associated with the affected limb (for example, making a fist and releasing it). The second type of lymphedema is acute and painful and occurs 4-6 weeks after surgery. This type of lymphedema is caused by swelling of the lymphatic vessels. It is treated by keeping the affected arm or leg raised and taking anti-inflammatory drugs. The third type of lymphedema is an acute form involving the skin, often occurring after an insect bite or minor injury or burn. It may occur on an arm or leg that is chronically swollen. This third form of edema is treated by keeping the arm or leg raised and by taking antibiotics. The fourth and most common form of lymphedema is slow and painless and usually occurs 18-24 months after surgery to remove a tumor or lymph nodes. If it develops later, it may mean that the tumor is coming back.

Acute lymphedema is a temporary condition that lasts less than 6 months. The skin indents when touched and stays indented, but there is no hardening of the skin. A patient may be more likely to develop lymphedema if he or she has surgical drains that leak protein into the surgical site; inflammation; no movement of the arm or leg; or a temporary lack of lymphatic function in one area of the body.

Chronic lymphedema is the hardest of all types of edema to treat. The damaged lymphatic system of the affected part of the body is not able to handle the increased fluid. This may happen when a tumor recurs or grows in an area of lymph nodes; when infection and/or injury of the lymphatic vessels occurs; when there is lack of movement of the arm or leg; after radiation therapy or surgery; with medical conditions such as diabetes, kidney problems, high blood pressure, congestive heart failure, or liver disease; or when preventive measures have not been taken after surgery. Lymphedema may also occur when cancer or cancer treatment causes loss of appetite, nausea, vomiting, depression, anxiety, or problems with metabolism.

A patient who is developing lymphedema will have soft, pitting edema that is helped by raising the arm or leg and by wearing elastic support garments. Continued problems with the lymphatic system cause fluid to flow back into the tissues, and the condition may become worse. This causes pain, heat, redness, and swelling as the body tries to get rid of the extra fluid. The skin becomes hard, stiff, and non-pitting and is no longer helped by raising the arm or leg or using elastic support.

Patients with lymphedema are more susceptible to infection. No effective treatment is yet available for patients with advanced chronic lymphedema. Edema may reoccur more easily in patients who have had lymphedema.

Risk factors

Factors that can lead to the development of lymphedema include radiation therapy to the area where the lymph nodes were surgically removed, problems after surgery that cause inflammation of the arm or leg, being overweight, the number of lymph nodes removed in surgery, and being elderly.

Patients who are at risk for lymphedema are those with:

- Breast cancer if they have received radiation therapy or had lymph nodes removed. Radiation to the underarm area after the lymph nodes have been removed increases the occurrence of lymphedema.

- Melanoma of the arms or legs if the patient has had lymph nodes removed from the underarm area and/or received radiation therapy.

- Prostate cancer treated by radiation therapy to the whole pelvis or by surgery.

- Cancer of the female reproductive tract that is advanced, treated with surgery to remove the lymph nodes, or treated with radiation therapy.

- Cancer that has spread to the lower abdomen, such as metastatic ovarian, testicular, colorectal, pancreatic, or liver cancer. The pressure from the growing tumor can destroy the lymphatic vessels and block lymphatic drainage.

Diagnosis

Specific criteria for diagnosing lymphedema do not yet exist. About half of patients with mild edema describe their affected arm or leg as feeling heavier or fuller than usual. To evaluate a patient for lymphedema, a history and physical examination of the patient should be completed. The history should include any past surgeries, problems after surgery, and the time between surgery and the onset of symptoms of edema. Any changes in the edema should be determined as well as any history of injury or infection. Knowing the medications a patient is taking is also important for diagnosis.

MANAGEMENT

Prevention Patients at risk for lymphedema should be identified early, monitored, and taught self-care. A patient may be more likely to develop lymphedema if he or she eats an inadequate diet, is overweight, is inactive, or has other medical problems. To detect the condition early, the following should be examined: weight, measurements of the arms and legs; protein levels in the blood; ability to perform activities of daily living; history of edema, previous radiation therapy, or surgery; and other medical illnesses, such as diabetes, high blood pressure, kidney disease, heart disease, or phlebitis (inflammation of the veins).

It is important that the patient know about his or her disease and the risk of developing lymphedema. Poor drainage of the lymphatic system due to surgery to remove the lymph nodes and/or radiation therapy may make the affected arm or leg more susceptible to serious infection. Even a small infection may lead to serious lymphedema.

Patients should understand the dangers of developing lymphedema and should be taught about arm, leg, and skin care after surgery and/or radiation therapy (see Table I below). It is important that this care continues for life, since lymphedema can occur 15 or more years after surgery. Breast cancer patients who follow instructions about skin care and proper exercise after mastectomy are less likely to experience lymphedema.

Lymphatic drainage is improved during exercise, therefore exercise is important in preventing lymphedema. Breast cancer patients should do hand and arm exercises after mastectomy. Patients who have surgery that affects pelvic lymph node drainage should do leg and foot exercises. The doctor determines how soon patients should start exercising after surgery. Physical therapists should develop an individual exercise program for the patient.

Better recovery occurs when lymphedema is discovered early, so patients should be taught to recognize the early signs of edema and to tell the doctor about any of the following symptoms: feelings of tightness in the arm or leg; rings or shoes that don't fit; weakness; pain, aching, or heaviness; redness, swelling, or signs of infection.

Patient Teaching Guide

1. Use an electric razor for shaving
-wear gardening and cooking gloves; use thimbles for sewing
-take care of nails; do not cut cuticles

2. Legs
-keep the feet covered when going in the ocean
-keep the feet clean and dry; wear cotton socks
-cut toenails straight across; see a podiatrist

3. Either arms or legs
-suntan gradually; use sunscreen
-clean cuts with soap and water, then use antibacterial ointment
-use gauze wrapping instead of tape
-talk to the doctor about any rashes
-avoid needle sticks of any type in the affected arm or leg
-avoid extreme hot or cold, such as, ice packs, heating pads
-do not overwork the affected arm or leg

4. Do not put too much pressure on the arm or leg:
-do not cross legs while sitting -wear loose jewelry; wear clothes without tight bands
-carry a handbag on the unaffected arm
-do not use blood pressure cuffs on the affected arm
-do not use elastic bandages or stockings with tight bands
-do not sit in one position for more than 30 minutes

5. Watch for signs of infection, such as redness, pain, heat, swelling, fever. Call the doctor immediately if any of these signs appear.

6. Do exercises regularly to improve drainage.

7. Keep regular follow-up appointments with the doctor.

8. Check all areas of the arms and legs every day for signs of problems:
-measure around the arm or leg periodically or if the limb seems swollen
-use a tape measure at two consistent places on the arm or leg
-tell the doctor if the limb suddenly gets larger

9. The arm or leg may be less sensitive. Use the unaffected limb to test temperatures for bath water or cooking.

10. Eat a well-balanced diet. 

Treatment

Lymphedema is treated by mechanical methods and with medication. Mechanical methods include raising the arm or leg, wearing custom-fitted clothes that apply controlled pressure, cleaning the skin carefully to prevent infection, and controlling body weight. When used, pressure garments should cover the entire area of edema. For example, a stocking that only reaches the knee can become tight and cause problems if there is edema in the thigh. Pumps on the arm and leg that give intermittent pressure are very helpful. The cuff is inflated and deflated according to a controlled time cycle. This increases fluid flow in the veins and lymphatic vessels and keeps fluid from collecting in the arm or leg.

Antibiotics may be used to treat and prevent infections. Other types of drugs such as diuretics or anticoagulants are generally not helpful, and may even worsen the problem.

Finding the exact cause of the swelling and treating it correctly is important. Edema often leads to infection, which then increases protein deposits in the tissues. If an infection is diagnosed, appropriate antibiotics should be given. Blood clots should be ruled out, since massage and therapy to encourage drainage may make the clots more dangerous. If blood clots are found, they should be treated with medication.

Coumarin was once used in some foods and medications in the United States. Coumarin was found to cause liver damage, and its use in foods and drugs in the United States has been banned since the 1950's.

Coumarin has been studied to determine if it is effective in reducing the swelling caused by lymphedema. Research has not shown coumarin to be an effective treatment for lymphedema and has shown that it may cause liver damage. Coumarin is available in some countries, but has not been approved for use in the United States.

Psychosocial considerations

Because lymphedema is disfiguring and sometimes painful and disabling, it can create mental, physical, and sexual problems. Women who develop lymphedema after treatment for breast cancer have more mental, physical, and sexual difficulties than women who do not develop lymphedema. The added stresses associated with lymphedema may interfere with its treatment that is often painful, difficult, and time-consuming.

Coping with lymphedema in the upper body after breast cancer treatment is especially difficult for patients who have little social support. Some patients may react to the problem by withdrawing. It is also difficult for patients with painful lymphedema. Patients with lymphedema may be helped by group and individual counseling that provides information about ways to prevent lymphedema, the role of diet and exercise, advice for picking comfortable and flattering clothes, as well as emotional support.

Other treatment

Surgery for lymphedema usually results in complications and is seldom recommended to the cancer patient.

COMPLICATIONS

A rare but fatal complication of lymphedema is lymphangiosarcoma, a tumor of the lymphatic vessels. The average time between mastectomy and the appearance of lymphangiosarcoma is about 10 years. After a patient develops lymphangiosarcoma, the average survival time is 1.3 years.

The cause of lymphangiosarcoma is not known. It appears as one or more bluish- red bumps on the affected arm or leg. First, one purple-red, slightly raised area in the skin of the arm or leg appears. The patient usually describes it as a bruise. Later, more tumors appear, and the bumps grow. Death usually results from metastases to the lungs.

TO LEARN MORE

For more information, call the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615. The call is free and a trained information specialist is available to answer your questions.

There are many other places to get information about cancer treatment and services. Check the social service office at your hospital for local and national agencies that can help with finances, getting to and from treatment, care at home, and dealing with other problems.

Write to the National Cancer Institute at this address:

National Cancer Institute Building 31, Room 10A24 9000 Rockville Pike Bethesda, MD 20892

Link no longer valid - Saints Memorial

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What is Lymphoma?

Lymphoma Information Network

www.lymphomainfo.net/

Lymphoma is a broad term encompassing a variety of cancers of the lymphatic system. The lymphatic system helps filter out bacteria and is important in fighting disease. Every so often, the lymph vessels widen into lymph nodes. Most of us have felt these under our arms or neck (like when sick with mono or an infection).

In lymphoma, some of the cells in the lymph system multiply uncontrollably - if all the cells are the same they are called malignant or cancerous. Lymphoma is differentiated by the type of cell that multiplies and how the cancer presents itself. 

The two main groups of lymphoma in humans are Hodgkin's Disease (characterized by the growth of Reed-Sternberg cells in the cancer) and the Non-Hodgkin's Lymphomas.  If you want to know the differences between these two groups, a chart is now available.

53,370 Americans (28,850 men and 25,520 women) are expected to be diagnosed with non-Hodgkin’s lymphoma in the US in 2004. About 7,880 new cases of Hodgkin's disease will be diagnosed in the United States this year. Hodgkin's lymphoma affects women somewhat less often than men. Of the 7,880 new cases, about 3,550 occurred in women, and 4,330 in men.

Since the early 1970s, incidence rates for non-Hodgkin’s lymphoma have nearly doubled. Incidence rates for Hodgkin’s disease have declined slightly over the same time period, especially among the elderly.  The rise of AIDS has also contributed to the number of people diagnosed as their immune system is weakened enough to get a number of illnesses.

Lymphoma is a serious condition. Lymphoma has indeterminate symptoms so it is sometimes hard to diagnose.  Unfortunately, other medical problems often appear as lymphoma symptoms (infections and "Cat Scratch Fever" are two). 

Animal lymphomas are not related to human cancers at all.

The best advice is to see a qualified doctor and get all the information you can about your condition as things develop. Use the resources listed as an aid in the quest for information. Informed people make better decisions.

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Non-Hodgkin Lymphoma

What is non-Hodgkin lymphoma?

Non-Hodgkin lymphoma (also known as non-Hodgkin's lymphoma, NHL, or sometimes just lymphoma) is a cancer that starts in cells called lymphocytes, which are part of the body's immune system. Lymphocytes are in the lymph nodes and other lymphoid tissues (such as the spleen and bone marrow). These will be described in more detail in the section "The lymph system and lymphoid tissue."

Some other types of cancer -- lung or colon cancers, for example -- can spread to lymph tissue such as the lymph nodes or bone marrow. But cancers that start in these places and then spread to the lymph tissue are not lymphomas.

There are 2 main types of lymphomas.

These 2 types of lymphomas differ in how they behave, spread, and respond to treatment.

Doctors can usually tell the difference between them by looking at the cancer cells under a microscope. In some cases, sensitive lab tests may be needed to tell them apart.

http://www.cancer.org/Cancer/Non-HodgkinLymphoma/DetailedGuide/non-hodgkin-lymphoma-what-is-non-hodgkin-lymphoma

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Lymphoma Links:

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The Furry Monkey

Another on of my very favorite Lymphoma sites - from the UK.  The best - a fellow lymphoma survivor's website.

http://www.thefurrymonkey.co.uk/

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Lymphoma.com

http://www.lymphoma.com//

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The Leukemia and Lymphoma Network

www.leukemia.org/

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Lymphoma Research Foundation

www.lymphoma.org//

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Cutaneous Lymphoma Foundation

http://www.clfoundation.org/

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Lymphoma-net.org

The information provided on lymphoma-net.org is not intended for residents of the USA

*As of 01/06/2012 site is under construction

http://lymphoma-net.org/

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The Lymphoma Association

http://www.lymphoma.org.uk//

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Non-Hodgkin's Lymphoma

National Cancer istitute

http://www.cancer.gov/cancertopics/types/non-hodgkin

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Lymphoma Foundation Canada

http://www.lymphoma.ca/

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Mantle Cell Lymphoma Consortium

http://www.mantlecelllymphoma.org/

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Additional links located at:

http://www.cancerlinks.org/lymphoma.html

PubMed NIH Abstracts

http://www.ncbi.nlm.nih.gov/sites/entrez

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What Is Non-Hodgkin Lymphoma?

Non-Hodgkin lymphoma (non-Hodgkin’s lymphoma, or NHL) is cancer, sometimes called lymphoma, that starts in lymphoid tissue (also called lymphatic tissue), which is part of the lymphatic system.

Lymph nodes make and store lymphocytes, infection-fighting white blood cells. They are connected throughout the body by narrow tubes similar to blood vessels called lymph vessels. These lymph vessels carry a colorless, watery fluid (lymphatic fluid) that contains lymphocytes. Eventually the lymphatic fluid is emptied into a major blood vessel in the left upper chest.

Other types of cancer - lung or colon cancers, for example - can develop in other organs and then spread to lymphoid tissue. But cancers that spread to the lymph nodes are not lymphomas. Lymphomas start from lymphocytes in either the lymphoid tissue or lymphoid organs and can spread from there.

There are 2 main types of lymphomas. Hodgkin lymphoma (also known as Hodgkin's lymphoma, Hodgkin disease, or Hodgkin's disease) is named after Dr. Thomas Hodgkin, who first described it as a new disease in 1832. All other types of lymphoma are called non-Hodgkin lymphomas.

These 2 types of lymphoma can usually be distinguished from each other by examining the cancerous tissue under a microscope. In some cases, more tests to identify specific chemical components of the lymphoma cells may be needed. Hodgkin disease is discussed in a separate American Cancer Society document.

Lymphoid Tissue

Lymphoid tissue is formed by several types of immune system cells that work together to resist infections. Lymphoid tissue is found in many places throughout the body. These are described below.

The main cell type found in lymphoid tissue is the lymphocyte. The 2 main types of lymphocytes are B lymphocytes (B cells) and T lymphocytes (T cells). Although both types can develop into lymphoma cells, B-cell lymphomas are much more common than T-cell lymphomas. These 2 types account for 85% (B-cell) and 15% (T-cell) of cases of non-Hodgkin lymphoma.

Normal T cells and B cells do different jobs within the immune system.

B cells normally help protect the body against germs (bacteria or viruses) by producing proteins called antibodies. The antibodies attach to the bacteria or viruses and attract immune system cells that surround and digest the antibody-coated germs. Antibodies also attract certain blood proteins that can kill bacteria.

There are several types of T cells, each with a specialized job. Some normal T cells help protect the body against viruses, fungi, and some bacteria. They recognize specific substances found in virus-infected cells and destroy these cells. T cells can also release substances called cytokines that attract certain other types of white blood cells, which then digest the infected cells. T cells are also thought to destroy some types of cancer cells, as well as the cells of transplanted organs. Some types of T cells play a role in stimulating or inhibiting the activity of other immune system cells.

Normal B cells and T cells can be recognized by laboratory tests that identify certain distinctive substances on their surfaces. Certain substances are found only on B cells, and others are found only on T cells.

There are also several stages of B-cell and T-cell development that can

There are also several stages of B-cell and T-cell development that can be recognized.

This information on types of lymphocytes is helpful because each type of lymphoma tends to resemble a particular subtype of normal lymphocyte at a certain level of development. Determining the type of lymphoma a person has is the first step in considering treatment options.

Organs That Contain Lymphoid Tissue

Because lymphatic tissue is found in many parts of the body, lymphomas can start almost anywhere. The major sites of lymphatic tissue are listed below.

Lymph nodes are bean-sized organs located throughout the body and connected by a system of lymphatic vessels. These vessels are like veins, except that instead of carrying blood, they carry lymph (a clear fluid containing waste products and excess fluid from tissues) and immune system cells traveling to lymph nodes from other tissues.

Lymph nodes increase in size when they fight an infection. Lymph nodes that grow in reaction to infection are called reactive nodes or hyperplastic nodes and are often tender to the touch. An enlarged lymph node is not usually serious. Enlarged lymph nodes in the neck are often felt in people with sore throats or colds. But a large lymph node is also the most common sign of lymphoma. Lymph node enlargement is discussed more in the section "How Is Non-Hodgkin Lymphoma Diagnosed?".

The spleen is found under the lower part of the rib cage on the left side of the body. An average adult spleen weighs about 5 ounces. The spleen produces lymphocytes and other immune system cells to help fight infection. It stores healthy blood cells and filters out damaged blood cells, bacteria, and cell waste.

The thymus gland is located in the front of the chest at the base of the neck. Before birth, the thymus plays a vital role in development of T lymphocytes. The thymus gland's size (about 1 ounce) and function diminish over the first 20 years of life. Although its size and activity decline with age, the thymus continues to be active in immune system function throughout life.

Adenoids and tonsils are collections of lymphoid tissue located at the back of the throat. They produce antibodies against germs that are breathed in or swallowed. They are easy to see when they become enlarged during an infection or if they become cancerous.

The stomach and intestinal tract as well as many other organs also contain some lymphatic tissue.

The bone marrow (the soft inner part of bones) produces red blood cells, blood platelets, and white blood cells. Red blood cells carry oxygen from the lungs to the rest of the body. Platelets plug up small holes in blood vessels caused by cuts or scrapes. White blood cells’ main job is fighting infections. The 2 main types of white blood cells are granulocytes and lymphocytes. Bone marrow lymphocytes are B cells. Sometimes lymphomas start from bone marrow lymphocytes.

Classification of Non-Hodgkin Lymphoma

The classification of non-Hodgkin lymphoma seems quite confusing (even for many doctors) because there are so many types and because several different systems of classification have been developed. The most recent system is the Revised European-American Lymphoma/World Health Organization (REAL/WHO) classification.

The reason for the changes in classification is that the old systems of classifying lymphomas by their appearance alone no longer worked. Modern science had discovered many chemical and genetic characteristics that identified lymphoma cells better than did their appearance. As we learn more about genes in humans, the genetic changes in lymphomas will play a larger role in classification, and new systems will be developed.

REAL/WHO system:

The REAL/WHO system not only uses the appearance of the lymphoma cells for classification but also uses chromosome changes of the cells and their chemistry. The chemistry is mainly evaluated by looking for certain proteins on the surface of the cells. This overview classifies the most common lymphomas according to whether they are B-cell or T-cell lymphomas and lists them by how common they are.

B-Cell Lymphomas

Diffuse large B-cell lymphoma (DLBCL): This kind makes up about 31% of all lymphomas. The cells are large when viewed under the microscope. This is a fast growing lymphoma.

The usual symptoms are a quickly growing mass in the body or in a lymph node that you can feel. Although this lymphoma usually starts in lymph nodes, it can grow in other areas such as the intestines, bone, and the brain or spinal cord.

About one third of these lymphomas are confined to one part of the body (localized). When it is localized this type of lymphoma is considered to be more curable than when it has spread to other parts of the body. Scientists using genetic testing of the lymphomas have found that there are 2 kinds. One kind has a very good outlook and responds well to treatment while the other doesn’t. In spite of these genetic differences the 2 types look alike under the microscope.

DLBCL can affect any age group but occurs mostly in older people. The average age is in the mid-60s. About 40% to 50% of people with this lymphoma are cured with therapy.

Follicular lymphoma:

About 22% of all lymphomas are follicular lymphomas. The term "follicular" is used because the cells tend to grow in a circular, or nodular, pattern in lymph nodes. This is a slow growing lymphoma.

Most of the time, this lymphoma occurs in many lymph node sites in the body, as well as in the bone marrow.

The average age for people with this lymphoma is about 60. It is rare in very young people. In advanced stages, it is not considered curable by standard treatment. But it is slow growing, and the 5-year survival rate (the percentage of people surviving at least 5 years) is around 60% to 70%. Over time, many follicular lymphomas change (transform) into a fast growing diffuse B-cell lymphoma.

Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL):

These related diseases account for 7% of all lymphomas. In both kinds, the same type of cell, the small lymphocyte, is involved. The only difference is in where the cancer occurs. In CLL it is mostly in the blood, whereas it is mainly in the lymph nodes in lymphoma. Both are slow growing diseases, although CLL, which is much more common, tends to be slower.

CLL and SLL are not considered curable with standard treatments. But depending on the stage and growth rate of the disease, patients can live well over 10 years with this lymphoma. Occasionally over time these slow growing lymphomas transform into a more aggressive type of lymphoma.

Mantle cell lymphoma:

About 6% of lymphomas are this kind. The cells are small to medium in size. It is usually widespread when it is diagnosed, involving lymph nodes, bone marrow, and, very often, the spleen.

Men are most often affected. The average age of patients is about 63. Although this isn’t a very fast growing lymphoma, it is a very serious one. Only 20% of patients survive at least 5 years.

Extranodal marginal zone B-cell lymphomas - mucosa-associated lymphoid tissue (MALT) lymphomas:

These account for about 8% of lymphomas. The cells are small.

Most MALT lymphomas arise in the stomach and are thought to stem from an infection by the bacteria Helicobacter pylori. Other possible sites are lung, skin, thyroid, salivary gland, and tissues surrounding the eye. Usually it is confined to the area where it began and is not widespread.

The average age for patients with this lymphoma is about 60. It is a slow growing lymphoma and is often curable in its early stages.

Nodal marginal zone B-cell lymphoma:

This kind accounts for about 2% of lymphomas. The cells are small. Mostly lymph nodes are involved, although the cells can also sometimes be found in the bone marrow.

This is a slow growing lymphoma, and many patients are cured if they are diagnosed in the early stages.

Splenic marginal zone B-cell lymphoma:

This is a rare lymphoma. The cells are small. Most often the lymphoma is found only in the spleen and bone marrow.

Patients are often elderly and male and suffer from fatigue and discomfort caused by an enlarged spleen. Because the disease is slow growing, treatment may not be necessary unless the symptoms become troublesome.

Primary mediastinal B-cell lymphoma: This type accounts for about 2% of all lymphomas. The cells are large. This lymphoma starts in the mediastinum (the area around the heart and behind the chest bone). It usually is localized at the beginning. It rarely involves the bone marrow. It can cause trouble breathing because it often presses into the air passages leading into the lung. It can also block the superior vena cava (the large vein that returns blood to the heart from the arms and head). This can cause the arms and face to swell.

About two thirds of people with this lymphoma are women. Most are young - in their 30s. It is a fast growing lymphoma but it is treatable. About half of patients can be cured. Genetic studies have shown that this type of lymphoma is closely related to Hodgkin disease.

Burkitt lymphoma: This type makes up about 2% of all lymphomas. It is named after the doctor who first described this disease in African children and young adults. The cells are medium sized. Another kind of lymphoma, called Burkitt-like lymphoma, has slightly larger cells. Because this second kind of lymphoma is hard to tell apart from Burkitt lymphoma, the REAL/WHO classification combines them.

This is a very fast growing lymphoma. In the African variety, it often starts as tumors of the jaws or other facial bones. In the more common types seen in the US, the lymphoma usually starts in the abdomen, where it forms a large tumor mass. It can also spread to the brain and spinal fluid.

Close to 90% of patients are male, and the average age is about 30.

Although this is a fast growing lymphoma, about half of patients are cured by aggressive chemotherapy.

Lymphoplasmocytic lymphoma (Waldenstrom macroglobulinemia): This kind is not common, accounting for 1% of lymphomas. The cells are small and found mainly in the bone marrow, lymph nodes, and spleen. It is slow growing. Most of the time the lymphoma cells produce an antibody called immunoglobulin M (IgM), which is a very large protein. This antibody circulates in the blood and causes it to thicken, leading to the symptoms typical of this disease.

Symptoms caused by high IgM levels include weakness, fatigue, problems with vision (due to poor circulation in blood vessels in the back of the eyes), a tendency to bleed easily, and neurological problems (such as headache, dizziness, and confusion) caused by poor blood flow within the brain. High blood IgM levels can also damage some organs, such as the kidneys.

Although this lymphoma isn’t curable, most patients live longer than 5 years.

For more information, see the ACS document "Waldenstrom Macroglobulinemia."

Hairy cell leukemia: This lymphoma is rare - about 500 people in the US are diagnosed with this type each year. The typical cell is small with projections around it that have a hair-like appearance. It typically is found in the bone marrow and spleen and circulating in the blood. It is slow growing, and some patients never need treatment. An enlarging spleen or dropping blood counts because of bone marrow replacement by lymphoma are the usual reasons to begin treatment. Patients are older in general.

Primary central nervous system (CNS) lymphoma: This lymphoma usually involves the brain (called primary brain lymphoma), but it also can be found in the spinal cord and in tissues around the spinal cord and the eye. Over time, it becomes widespread in the central nervous system. Although this was a rare tumor in the past, it has become more common in patients with acquired immune deficiency syndrome (AIDS). Most people develop headache and confusion. They can also have vision problems and, rarely, paralysis.

The outlook for people with this condition is poor. But about 30% of people can live at least 5 years with today's treatment.

T-Cell Lymphomas

Precursor T-lymphoblastic lymphoma/leukemia: This disease can be considered either a lymphoma or leukemia. The distinction depends on whether more or less than 25% of bone marrow cells are lymphoma cells. Leukemias have more than 25% involvement; lymphomas have less. Usually there is a mass of lymphoma in the mediastinum (the area around the heart and behind the chest bone).

About 2% of all lymphomas fall into this category. Patients are most often (75%) men, and their average age is about 25. The typical cell is small to middle sized. The lymphoma is fast growing, but if it hasn’t spread to the bone marrow when it is first diagnosed, the chance of cure with chemotherapy is quite good. Once it involves the bone marrow, only 20% of patients can be cured.

Peripheral T-cell lymphomas: There are several kinds of peripheral T-cell lymphomas, which, in total, account for about 7% of all lymphomas.

Page updated Jan. 6, 2012