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Reviewed by the Faculty of Harvard Medical School

Lymphedema

What Is It?

Lymphedema is the buildup of fluid called lymph in the tissues under your skin when something blocks its normal flow. This causes swelling, most commonly in an arm or leg.

Lymph normally does an important job for your body. It carries foreign material and bacteria away from your skin and body tissues, and it circulates infection-fighting cells that are part of your immune system. Lymph flows slowly through the network of vessels called your lymphatic system, stopping at points along the way to be filtered through lymph nodes (small bean-shaped organs that are part of your immune system). Lymph first collects by seeping out of your cells into the smallest lymphatic vessels near the skin. After treveling through these small vessels, lymph drains into deeper, wider lymph channels that run through the body. Eventually, lymph fluid returns to the blood.

Lymphedema is not the same as edema, which is another condition that causes arm or leg swelling. In both problems there is too much fluid in the limb, but only lymphedema results from blocked drainage. Without a blockage, the fluid can be pushed forward in your lymphatic system, so you can see pitting — small temporary indentations left on the skin after you press on the swollen area. Pitting does not happen when you press on skin if you have lymphedema.

In most cases of lymphedema, the lymphatic system has been injured so that the flow of lymph is blocked either temporarily or permanently. Common causes include:

  • Surgical damage — Surgical cuts and the removal of lymph nodes can interfere with normal lymph flow. Sometimes, lymphedema appears immediately after surgery and goes away quickly. In other cases, lymphedema develops from one month to 15 years after a surgical procedure. Lymphedema is most commonly seen in people who have had surgery for breast cancer. It develops in up to 25% of those who have a breast removed (mastectomy) along with the lymph nodes under the arm. The risk doubles for those who also receive radiation treatments to the underarm area. Lymphedema also may occur after surgery for prostate or testicular cancer, melanoma and cancer in the lower abdomen.
  • An infection involving the lymphatic vessels — Rarely, a bacterial infection that causes a red stripe on the arm or leg (lymphangitis) can be severe enough to cause lymphedema. In areas of the tropics and subtropics, such as South American, the Caribbean, Africa, Asia and the South Pacific, parasites commonly cause lymphedema. Filariasis, a parasitic worm infection, blocks the the lymph channels and causes swelling and thickening below the skin, usually in the legs. Filariasis rarely is seen in the United States, except in people who emigrated from tropical areas
  • Cancer — Lymphoma, a cancer that starts in the lymph nodes, or other types of cancer that spread to the lymph nodes may block lymph vessels.
  • Radiation therapy for cancer — This treatment can cause scar tissue to develop and block the lymphatic vessels.

When lymphedema occurs without any known injury or infection, it is called primary lymphedema. Doctors diagnose three types of primary lymphedema according to when symptoms first appear:

At birth – Also known as congenital lymphedema. Risk is higher in female newborns. The legs are affected more often than the arms. Usually both legs are swollen.

After birth but before age 36 – Usually, it is first noted during the early teenage years. This is the most common type of primary lymphedema.

Age 36 and older – This is the rarest type of primary lymphedema.

All three types of primary lymphedema are probably related to the abnormal development of lymph channels before birth. The difference is when in life they first cause swelling of the legs or arms.

Symptoms

Lymphedema causes swelling with a feeling of heaviness, tightness or fullness, usually in an arm or leg. In most cases, only one arm or leg is affected. Swelling in the leg usually begins at the foot, and then moves up if it worsens to include the ankle, calf and knee. Additional symptoms can include:
  • A dull ache in the affected limb
  • A feeling of tightness in the skin of the affected limb
  • Difficulty moving a limb or bending at a joint because of swelling and skin tightness
  • Shoes, rings or watches that suddenly feel too tight

Lymphedema can make it easier to develop a skin infection. Signs of infection include fever, pain, heat and redness. If lymphedema becomes chronic (long lasting), the skin in the affected area often becomes thickened and hard.

Diagnosis

Your doctor will ask you whether you have had any surgery, radiation treatments, or infections in the affected area. The doctor may ask if you have ever had a blood clot. If a child has lymphedema, the doctor will ask if anyone in your family had leg swelling starting at a young age. This may indicate an inherited disorder.

Your doctor will examine the swollen area and press on the affected skin to look for a fingertip indentation (pitting). Your doctor may measure the circumference of the affected arm or leg to determine how swollen it is compared to the other one. The doctor will look for signs of infection, including fever, redness, warmth and tenderness.

Usually, no specific testing is necessary to diagnose lymphedema. But tests may be ordered if there is no obvious cause for your condition:

  • A blood count can look for a high level of white cells, which means you might have an infection.
  • An ultrasound can look for blood clots, which can cause an arm or leg to swell.
  • A computed tomography (CT) scan looks for a mass or tumor that could be blocking lymph vessels in the swollen arm or leg.
Expected Duration

How long lymphedema lasts depends on its cause. If lymphedema develops immediately after surgery, it can clear up within one week as the swelling goes down and thearm or leg is elevated to allow better drainage. If surgery or radiation therapy produced long-term damage to the lymphatic system, lymphedema can become a long-term or recurring problem.

Prevention

After breast cancer or prostate cancer surgery, your doctor or physical therapist may advise that you do specific exercises once you have fully recovered from the surgery. Using your muscles can encourage the flow of lymph through small channels. After breast surgery, you are less likely to develop lymphedema if you can avoid having injections, intravenous (IV) lines, or blood drawn in the arm on the side of the surgery. Also, be sure to get prompt treatment if you think you may have a skin infection on the side of your surgery.

Wearing a compression stocking during the day can help to prevent leg swelling.

Treatment

The basic treatment for lymphedema includes:
  • Elevating the affected limb
  • Doing exercises to help reduce swelling
  • Keeping the affected limb clean and dry and periodically applying lubricating lotions.

If lymphedema affects your legs, avoid wearing socks with tight bands across the top. Avoid standing for long periods. If you work on your feet or at a desk all day, your doctor may prescribe special compression stockings for you to wear throughout the day. Your doctor may suggest that you follow a protein-rich, low-salt diet and that you lose weight if you are obese.

For people with more severe lymphedema, doctors prescribe inflatable sleeves that can be worn around the arm or leg, called pneumatic compression devices. These sleeves are attached to a machine that alternately fills and deflates them with air, and they can be used at home to help reduce limb swelling. An alternative to air-filled sleeves is to wrap the limb with a non-elastic bandage, and adjust the bandage each time the swelling decreases.

A very helpful treatment is a type of massage therapy called manual lymph drainage. Massage should not be done if you have cancer in the limb. People with lymphedema are more prone to infection in the affected arm or leg. If your doctor suspects you have an infection, you will need to take antibiotics by mouth or into a vein (intravenously).

When To Call a Professional

Make an appointment with your doctor if you develop symptoms of lymphedema in an arm or leg.

You should call your doctor the same day if your have symptoms that could be from an infection:

  • Fever, redness, warmth or increased pain in addition to swelling
  • Open sores or areas of broken skin
Prognosis

It isn't always easy to predict whether edema will last. Most of the time, treatments can improve lymphedema symptoms.

Additional Info

National Heart, Lung and Blood Institute (NHLBI)
P.O. Box 30105
Bethesda, MD 20824-0105
Phone: 301-592-8573
TTY: 240-629-3255
Fax: 301-592-8563
Email: nhlbiinfo@rover.nhlbi.nih.gov
http://www.nhlbi.nih.gov/

National Cancer Institute (NCI)
U.S. National Institutes of Health
Public Inquiries Office
Building 31, Room 10A03
31 Center Drive, MSC 8322
Bethesda, MD 20892-2580
Phone: 301-435-3848
Toll-Free: 1-800-422-6237
TTY: 1-800-332-8615
Email: cancergovstaff@mail.nih.gov
http://www.nci.nih.gov/

National Lymphedema Network
Latham Square, 1611 Telegraph Ave.
Suite 1111
Oakland, CA 94612-2138
Toll-Free: 1-800-541-3259
Phone: 510-208-3200
Fax: 510-208-3110
Email: nln@lymphnet.org
http://www.lymphnet.org/

Office Of Rare Diseases
National Institutes of Health
6100 Executive Blvd.
Room 3B01, MSC 7518
Bethesda, MD 20892-7518
Phone: 301-402-4336
Fax: 301-480-9655
Email: ord@od.nih.gov
http://rarediseases.info.nih.gov/

Aetna Members: If you are a female member of an HMO-based plan and would like more information about breast cancer and breast cancer prevention, please call 1-888-322-8742.

Miembros de Aetna: Si tiene un plan HMO y desea más información sobre el cáncer de seno y la prevención del cáncer de seno, por favor llame al 1-888-322-8742.

Last updated December 12, 2007




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