Lymphedema, also called lymphatic * obstruction, is swelling, usually of an arm or leg, due to the accumulation of fluids and other substances in soft tissues of the body. Lymphedema is caused by a blockage or malfunction in the body's lymphatic system. It is a common complication of surgery or radiation therapy * for treating cancer.
Primary lymphedemas are rare inherited disorders in which components of the lymphatic system are abnormal or absent. Most lymphedemas are secondary or acquired, usually as a result of surgery or trauma * resulting in a malfunction in the lymphatic system. Secondary lymphedema can be acute (temporary), lasting less than six months, or chronic, lasting six months or longer—perhaps for the remainder of one's life.
Lymphedema can occur anywhere in the body, but usually affects an arm or leg. What area is affected is determined by the cause of the lymphedema. Lymph-node removal to treat breast cancer can result in lymphedema in the arm. Treatments for gynecological cancer may result in lower-limb lymphedema.
The lymphatic system is a network of vessels and nodes located throughout the body that collects excess fluid and other substances from tissues and transports them to the circulatory system * . The walls of the lymphatic vessels take up water, proteins, fats, cell waste products, and foreign * substances, including bacteria * and viruses * . Valves in the vessels keep the clear lymphatic fluid, or lymph, flowing through progressively larger vessels in the direction of the heart. Lymph from the right arm and right side of the head and chest flows to the right lymphatic duct and is released into the large vein * under the right collarbone. The left lymphatic, or thoracic, duct collects fluid from the legs, the left arm, and left side of the head and chest and empties into the large vein under the left collarbone.
The lymphatic system is an integral part of the body's immune defenses. Lymph carries lymphocytes, or white blood cells, and other immune system * cells for fighting infection and disease. The fluid passes through lymph nodes—small bean-shaped structures that filter out harmful substances, recognize infectious organisms, and initiate an immune response.
If the volume of the lymphatic fluid exceeds the capacity of the system, protein-rich fluid collects in the tissues of the affected region. The accumulating fluid increases the size and number of channels in the tissues, which swell and begin to harden. The accumulating fluid also reduces the availability of oxygen, interferes with healing, and creates a suitable environment for bacterial infection.
There are four types of acute lymphedema:
Failure to control lymphedema in its early stages can lead to a chronic condition. Chronic lymphedema occurs when the lymphatic system cannot meet the increased requirement for fluid drainage from body tissues. As the lymphatic vessels expand, the fluid flows back into the tissues, worsening the condition.
Secondary lymphedema usually results from physical damage to the lymphatic system, most often the lymph nodes in the groin, pelvis, and/or armpits. Scar tissue resulting from surgery and/or radiation treatments for cancer, especially breast or testicular cancers, can disrupt the flow of lymphatic fluid and is the most common cause of lymphedema. The removal of a large number of lymph nodes or radiation to an area where lymph nodes have been removed increases the risk of lymphedema. Poor drainage of the lymphatic system following removal of lymph nodes or radiation therapy can also lead to infection, and even minor infections can cause serious lymphedema.
In addition to surgeries for breast and testicular cancers, lymphedema is a possible complication of any surgery in which lymph nodes are removed, including surgeries for the following:
Lymphedema is both underdiagnosed and underreported. It has been estimated that about three million Americans are affected by secondary lymphedema. It has also been estimated that 20 to 40 percent of cancer survivors—especially the elderly—develop lymphedema at some point.
Lymphedema of the arm occurs in about 10 to 15 percent of women who have full axillary lymph node surgery for breast cancer (removing between 5 and 30 lymph nodes in the area). It occurs in only about 1 percent of women who are treated with a sentinel lymph node biopsy *
Approximately10 percent of gynecological cancer survivors are diagnosed with lymphedema, and another 15 percent reported lower-limb swelling. Lymphedema had been diagnosed in 36 percent of vulvar * cancer survivors.
The classic symptoms of lymphedema are discoloration and swelling of the affected region. The severity of the symptoms depends on the type of lymphedema:
Symptoms of gradual-onset acute lymphedema, the most common form, can include:
Chronic lymphedema is characterized by severe and painful swelling, heat, and redness. Chronic lymphedema can be distinguished from temporary lymphedema by the texture of the skin. With temporary lymphedema, pressing on the skin of the affected area with the fingertips leaves an indentation. With chronic lymphedema, the skin is hard and stiff and does not indent.
Lymphedema is often first diagnosed by a swelling or feeling of heaviness in the arm or leg. In its early stages, diagnostics may include the following:
Diagnostic imaging techniques may include the following:
Lymphedema is sometimes categorized as grades 1 through 4, with 4 being the most serious. It may also be classified in stages:
Appropriate treatment requires identifying the cause of the lymphedema. It is most often treated by physical means and/or medications. In its earliest stages, the swelling can be relieved by supporting the limb in a raised position, exercising it gently, and wearing elastic support garments. Acute lymphedema following surgery or injury can usually be relieved within a week by keeping the affected limb raised and contracting the muscles, such as repeatedly making a fist with the hand of an affected arm.
Other physical methods of treating lymphedema include:
Complex physical therapy or complex decongestive therapy (CDT) uses a combination of these methods. Some specialists believe that CDT should not be used on patients with recurrent or metastatic cancers due to the risk of spreading the cancer through the lymphatic system. The presence of blood clots must be ruled out before using manual lymphatic drainage, which could move clots toward the heart or lung. Some physicians believe that compression pumps can actually worsen the lymphedema.
Drug treatments for lymphedema include the following:
Repeated episodes of lymphedema, which stretch body tissues, increase the likelihood of recurrence. There is no effective treatment for advanced chronic lymphedema. Physical treatments provide no relief. Although surgery can reduce severe swelling, it cannot cure lymphedema. Reconstructive or debulking surgery is sometimes used to remove excess tissue in stage 3 lymphedema. However, because lymphedema surgery usually results in complications, it is rarely used on cancer patients.
Lymphedema can cause tissue damage by interfering with the uptake of nutrients * and by preventing limb mobility. Other complications of lymphedema include:
Chronic lymphedema often leads to lymphangitis * —an inflammation of the lymphatic vessels that affects the connective tissue * * and infections become chronic. These complications are irreversible. In addition to these physical challenges, lymphedema can be so disfiguring, painful, and disabling that mental and sexual problems arise.
Risk factors for lymphedema include:
Methods for preventing or controlling lymphedema include:
See also Filariasis • Immune System and Other Body Defenses: Overview
Zuther, Joachim Ernst, and Steve Norton. Lymphedema Management: The Comprehensive Guide for Practitioners, 3rd ed. New York: Thieme Publishing, 2013.
National Cancer Institute. “Lymphedema–Patient Version (PDQ®)” http://www.cancer.gov/about-cancer/treatment/side-effects/lymphedema/lymphedema-pdq (accessed May 12, 2016).
Vascular Disease Foundation. “Lymphedema.” http://vasculardisease.org/flyers/lymphedema-flyer.pdf (accessed May 12, 2016).
National Cancer Institute. 9609 Medical Center Dr., Bldg. 9609, MSC 9760, Bethesda, MD 20892-9760. Toll-free: 800-422-6237. Website: http://www.cancer.gov (accessed March 17, 2016).
National Lymphedema Network. 225 Bush St., Suite 357, San Francisco, CA 94104. Telephone: 415-908-3681. Website: http://www.lymphnet.org/ (accessed March 17, 2016).
* lymphatic (lim-FA-tik) means relating to the system of vessels and other structures that carry lymph, a colorless fluid, throughout the body's tissues; the lymphatic system plays an important role in protecting the body from infections.
* radiation therapy is a treatment that uses high-energy radiation from x-rays and other sources to kill cancer cells and shrink cancerous growths.
* trauma refers to a wound or injury, whether psychological or physical.
* circulatory system (SIR-kyoo-luh-tor-ee) is the system composed of the heart and blood vessels that moves blood throughout the body.
* foreign means coming from outside a person's body.
* bacteria (bak-TEER-ee-a) are single-celled microorganisms that typically reproduce by cell division. Some, but not all, types of bacteria can cause disease in humans. Many types can live in the body without causing harm.
* viruses (VY-rus-sez) are tiny infectious agents that can cause infectious diseases. A virus can only reproduce within the cells it infects.
* vein is a vessel that carries blood to the heart.
* vascular refers to veins and arteries (the blood vessels).
* congenital (kon-JEH-nih-tul) means present at birth.
* malformation (mal-for-MAYshun) is an abnormal formation of a body part.
* puberty (PU-ber-tee) is the period during which sexual maturity is attained.
* immune system (im-YOON) is the system of the body composed of specialized cells and the substances they produce that helps protect the body against disease-causing germs.
* inflammation (in-fla-MAY-shun) is the body's reaction to irritation, infection, or injury that often involves swelling, pain, redness, and warmth.
* prostate (PRAH-state) is a male reproductive gland located near where the bladder joins the urethra. The prostate produces the fluid part of semen.
* bladder (BLAD-er) is the organ that stores urine produced by the kidneys prior to discharge from the body.
* colon (KO-lin), also called the large intestine, is a muscular tube through which food passes as it is digested, just before it moves into the rectum and out of the body through the anus.
* tumors (TOO-morz) are abnormal growths of body tissue that arise from uncontrolled cell growth and have no physiological function. Tumors may or may not be cancerous.
* biopsy (BI-op-see) is a test in which a small sample of skin or other body tissue is removed and examined for signs of disease.
* vulvar (VUL-var) means related to the organs of the female genitals that are located on the outside of the body.
* edema (e-DEE-ma) means swelling in the body's tissues caused by excess fluids.
* obesity (o-BEE-si-tee) is an excess of body fat. People are considered obese if they weigh more than 30 percent above what is healthy for their height.
* diabetes (dye-uh-BEE-teez) is a condition in which the body's pancreas does not produce enough insulin or the body cannot effectively use the insulin made by the pancreas, resulting in increased levels of sugar in the blood.
* hypertension (HI-per-ten-chen) is abnormally high arterial blood pressure.
* kidney is one of the pair of organs that filter blood and remove waste products and excess water from the body in the form of urine.
* heart disease is a broad term that covers many conditions that prevent the heart from working properly to pump blood throughout the body.
* phlebitis (fle-BY-tis) refers to inflammation of a vein.
* MRI, short for magnetic resonance imaging, produces computerized images of internal body tissues based on the magnetic properties of atoms within the body.
* CT scans, computed tomography (to-MOG-ra-fee) scans, or CAT (computerized axial tomography) scans, use computers to view structures inside the body.
* ultrasound, also called a sonogram, is a diagnostic test in which sound waves passing through the body create images on a computer screen.
* blood clot is a thickening of the blood into a jellylike substance that helps stop bleeding. Clotting of the blood within a blood vessel can lead to blockage of blood flow.
* antibiotics (an-tee-by-AH-tiks) are drugs that kill or slow the growth of bacteria.
* nutrients are the components of food (protein, carbohydrate, fat, vitamins, and minerals) needed for growth and maintenance of the body.
* constipation is the sluggish movement of the bowels, usually resulting in infrequent, hard stools.
* opioid (O-pee-oyd) is a synthetic narcotic used to treat pain, such as morphine, codeine, or oxycodone.
* lymphangitis (lim-fan-JIE-tis) is inflammation of the lymphatic system, the system that carries lymph through the body. Lymph is a clear fluid that contains white blood cells.
* connective tissue helps hold the body together and is found in skin, joints, and bones.
* ulcers are open sores on the skin or the lining of a hollow body organ, such as the stomach or intestine. They may or may not be painful.