Acute versus gradual-onset lymphedema
Temporary versus chronic lymphedema
Risk factors
Diagnosis

The lymphatic system consists of a network of specialized lymphatic vessels and various tissues and organs throughout the body that contain lymphocytes (white blood cells) and other cells that help the body fight infection and disease. The lymphatic vessels are similar to veins but have thinner walls. Some of these vessels are very close to the skin surface and can be found near veins; others are just under the skin and in the deeper fatty tissues near the muscles and can be found near arteries. Muscles and valves within the walls of the lymphatic vessels near the skin surface help pick up fluid and proteins from tissues throughout the body and move the lymph in one direction, toward the heart. Lymph is slowly moved through larger and larger lymphatic vessels and passes through small bean-shaped structures called lymph nodes. Lymph nodes filter substances that can be harmful to the body and contain lymphocytes and other cells that activate the immune system to fight disease. Eventually, lymph flows into one of two large ducts in the neck region. The right lymphatic duct collects lymph from the right arm and the right side of the head and chest, emptying it into the large vein under the right collarbone. The left lymphatic duct or thoracic duct collects lymph from both legs, the left arm, and the left side of the head and chest, emptying it into the large vein under the left collarbone.

The lymphatic system collects excess fluid and proteins from the body tissues and carries them back to the bloodstream. Proteins and substances too big to move through the walls of veins can be picked up by the lymphatic vessels because they have thinner walls. Edema may occur when there is an increase in the amount of fluid, proteins, and other substances in the body tissues because of problems in the blood capillaries and veins or a blockage in the lymphatic system.

Lymphedema may be either primary or secondary. Primary lymphedema is a rare inherited condition in which lymph nodes and lymph vessels are absent or abnormal. Secondary lymphedema can be caused by a blockage or cut in the lymphatic system, usually the lymph nodes in the groin area and the armpit. Blockages may be caused by infection, cancer, or scar tissue from radiation therapy or surgical removal of lymph nodes. Lymphedema that is secondary to cancer is not usually life-threatening. This summary discusses secondary lymphedema.

There are four types of acute lymphedema. The first type of acute lymphedema is mild and lasts only a short time, occurring a few days after surgery to remove the lymph nodes or after injury to the lymphatic vessels or veins just under the collarbone. The affected limb may be warm and slightly red, but is usually not painful and gets better within a week by keeping the affected arm or leg supported in a raised position and by contracting the muscles in the affected limb (for example, making a fist and releasing it). The second type of acute lymphedema occurs 6 to 8 weeks after surgery or during a course of radiation therapy. This type may be caused by inflammation of either lymphatic vessels or veins. The affected limb is tender, warm or hot, and red and is treated by keeping the limb supported in a raised position and taking anti-inflammatory drugs. The third type of acute lymphedema occurs after an insect bite, minor injury, or burn that causes an infection of the skin and the lymphatic vessels near the skin surface. It may occur on an arm or leg that is chronically swollen. The affected area is red, very tender, and hot and is treated by supporting the affected arm or leg in a raised position and taking antibiotics. A compression pump should not be used and the affected area should not be wrapped with elastic bandages during the early stages of infection. Mild redness may continue after the infection. The fourth and most common type of acute lymphedema develops very slowly and may become noticeable 18 to 24 months after surgery or not until many years after cancer treatment. The patient may experience discomfort of the skin; aching in the neck, shoulders, spine, or hips caused by stretching of the soft tissues or overuse of muscles; or posture changes caused by increased weight of the arm or leg.

Temporary lymphedema is a condition that lasts less than 6 months. The skin indents when pressed 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 one of the following:

• Surgical drains that leak protein into the surgical site.
• Inflammation.
• Inability to move the limb(s).
• Temporary loss of lymphatic function.
• Blockage of a vein by a blood clot or inflammation.

Chronic (long-term) lymphedema is the most difficult of all types of edema to treat. The damaged lymphatic system of the affected area is not able to keep up with the increased need for fluid drainage from the body tissues. This may be caused by one of the following:

• Recurrence or spread of a tumor to the lymph nodes.
• Infection of and/or injury to the lymphatic vessels.
• Periods of not being able to move the limbs.
• Radiation therapy or surgery.
• Inability to control early signs of lymphedema.
• Blockage of a vein by a blood clot.

A patient who is in the early stages of developing lymphedema will have swelling that indents with pressure and stays indented but remains soft. The swelling may easily improve by supporting the arm or leg in a raised position, gently exercising, and wearing elastic support garments. Continued problems with the lymphatic system cause the lymphatic vessels to expand, allowing lymph to flow back into the body tissues and make the condition worse. Pain, heat, redness, and swelling result as the body tries to get rid of the extra fluid. The skin becomes hard and stiff and no longer improves with raised support of the arm or leg, gentle exercise, or elastic support garments.

Patients with chronic lymphedema are at increased risk of infection. No effective treatment is yet available for patients who have advanced chronic lymphedema. Once the body tissues have been repeatedly stretched, lymphedema may recur more easily.

Factors that can lead to the development of lymphedema include radiation therapy to an area where the lymph nodes were surgically removed, problems after surgery that cause inflammation of the arm or leg, a larger number of lymph nodes removed in surgery, and being older. The part of the body treated may affect where lymphedema occurs. For example, lymphedema in the arms may occur after treatment for breast cancer and lymphedema in the legs may occur after treatment for vulvar cancer.

Risk factors for lymphedema include the following:

• Breast cancer, if the patient received radiation therapy or had lymph nodes removed. Radiation therapy to the underarm area after surgical removal of the lymph nodes and having a larger number of lymph nodes removed increases the risk of lymphedema.



• Surgical removal of lymph nodes in the underarm, groin, or pelvic regions.



• Radiation therapy to the underarm, groin, pelvic, or neck regions.



• Scar tissue in the lymphatic ducts or veins and under the collarbones, caused by surgery or radiation therapy.



• Cancer that has spread to the lymph nodes in the neck, chest, underarm, pelvis, or abdomen.



• Tumors growing in the pelvis or abdomen that involve or put pressure on the lymphatic vessels and/or the large lymphatic duct in the chest and block lymph drainage.



• Having an inadequate diet or being overweight. These conditions may delay recovery and increase the risk for lymphedema.

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 medical history and physical examination of the patient should be completed. The medical 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 should any history of injury or infection. Knowing which medications a patient is taking is also important for diagnosis.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Not normal. An abnormal lesion or growth may be cancerous, premalignant (likely to become cancer), or benign.

In biology, to stimulate a cell in a resting state to become active. This causes biochemical and functional changes in the activated cell.

Symptoms or signs that begin and worsen quickly; not chronic.

Having to do with reducing inflammation.

A drug used to treat infections caused by bacteria and other microorganisms.

A blood vessel that carries blood from the heart to tissues and organs in the body.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A mass of blood that forms when blood platelets, proteins, and cells stick together. When a blood clot is attached to the wall of a blood vessel, it is called a thrombus. When it moves through the bloodstream and blocks the flow of blood in another part of the body, it is called an embolus.

Cancer that forms in tissues of the breast, usually the ducts (tubes that carry milk to the nipple) and lobules (glands that make milk). It occurs in both men and women, although male breast cancer is rare.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The smallest type of blood vessel. A capillary connects an arteriole (small artery) to a venule (small vein) to form a network of blood vessels in almost all parts of the body. The wall of a capillary is thin and leaky, and capillaries are involved in the exchange of fluids and gases between tissues and the blood.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

A disease or condition that persists or progresses over a long period of time.

A machine used to keep blood and lymph flowing by pushing air through bands or sleeves that are placed on the arms or legs.

The process of identifying a disease, such as cancer, from its signs and symptoms.

The things a person eats and drinks.

In medicine, to remove fluid as it collects; or, a tube or wick-like device used to remove fluid from a body cavity, wound, or infected area.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

In medicine, a tube or vessel of the body through which fluids pass.

Swelling caused by excess fluid in body tissues.

Liquid.

The area where the thigh meets the abdomen.

The complex group of organs and cells that defends the body against infections and other diseases.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

Redness, swelling, pain, and/or a feeling of heat in an area of the body. This is a protective reaction to injury, disease, or irritation of the tissues.

Transmitted through genes that have been passed from parents to their offspring (children).

The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

The flow of lymph from an area of tissue into a particular lymph node.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymph vessel.

A condition in which excess fluid collects in tissue and causes swelling. It may occur in the arm or leg after lymph vessels or lymph nodes in the underarm or groin are removed or treated with radiation.

A type of white blood cell. Lymphocytes have a number of roles in the immune system, including the production of antibodies and other substances that fight infections and other diseases.

A part of the body that performs a specific function. For example, the heart is an organ.

Being too heavy for one’s height. Excess body weight can come from fat, muscle, bone, and/or water retention. Being overweight does not always mean being obese.

Having to do with the pelvis (the lower part of the abdomen located between the hip bones).

The lower part of the abdomen, located between the hip bones.

An exam of the body to check for general signs of disease.

A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.

To come back or to return.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrent cancer.

Something that may increase the chance of developing a disease. Some examples of risk factors for cancer include age, a family history of certain cancers, use of tobacco products, certain eating habits, obesity, lack of exercise, exposure to radiation or other cancer-causing agents, and certain genetic changes.

Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spine encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spinal column, and vertebral column .

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

Having to do with the chest.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A blood vessel that carries blood to the heart from tissues and organs in the body.

Cancer of the vulva (the external female genital organs, including the clitoris, vaginal lips, and the opening to the vagina).

Refers to a blood cell that does not contain hemoglobin. White blood cells include lymphocytes, neutrophils, eosinophils, macrophages, and mast cells. These cells are made by bone marrow and help the body fight infections and other diseases. Also called WBC.