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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 and empties into the large vein under the right collar bone. The
left lymphatic duct or thoracic duct collects lymph from both legs, the
left arm and the left side of the head and chest and empties into the large
vein under the left collar bone.
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 arm pit. Blockages may be caused by infection, cancer,
or scar tissue from radiation therapy or surgical removal of lymph nodes.
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 within a few days after surgery to remove the lymph nodes or
injury to the lymphatic vessels or veins just under the collar bone. The
affected limb may be warm and slightly red, but is usually not painful
and gets better within a week after 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. Use of a compression pump or wrapping the affected
area with elastic bandages should not be done 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 or
aching in the neck and shoulders or spine and hips caused by stretching
of the soft tissues, 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:
- Surgical drains that leak protein into the surgical site.
- An inability to move the limb(s).
- Temporary loss of lymphatic function.
- Blockage of a vein by a blood clot or inflammation of a vein.
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 happen:
- After a tumor recurs or spreads to the lymph nodes.
- After an infection and/or injury of the lymphatic vessels.
- After periods of not being able to move the limbs.
- After radiation therapy or surgery.
- When early signs of lymphedema have not been able to be controlled.
- When a vein is blocked 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 and lymph flows back into the body tissues,
making the condition worse. This causes pain, heat, redness, and swelling
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, the number of lymph nodes removed in surgery, and being
Patients who are at risk for lymphedema
are those with:
- Breast cancer if they have received radiation therapy or
had lymph nodes removed. Radiation therapy to the underarm area after
surgical removal of the lymph nodes and the 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, under the collar bones, caused by
surgery or radiation therapy.
- Cancer that has spread to the lymph nodes in the neck, chest, underarm, pelvis,
- 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
- An inadequate diet or those who are 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 well as any history of injury or infection. Knowing the medications
a patient is taking is also important for diagnosis.
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:
- Comparison of actual weight to ideal 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.
- 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 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
be taught about arm, leg, and skin care after surgery and/or radiation
(see Considerations for Teaching Patients Prevention and Control of Lymphedema
list below). It is important that patients take precautions to prevent
injury and infection in the affected arm or leg, since lymphedema can occur
30 or more years after surgery. Breast cancer patients who follow instructions
about skin care and proper exercise after mastectomy are less likely to
Lymphatic drainage is improved during exercise,
therefore exercise is important in preventing lymphedema. Breast cancer
patients should do hand and arm exercises as instructed after mastectomy.
Patients who have surgery that affects pelvic lymph node drainage should
do leg and foot exercises as instructed. The doctor decides how soon patients
should start exercising after surgery. Physiatrists (doctors who specialize
in Physical Medicine and Rehabilitation) or physical therapists should
develop an individualized 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 become tight.
- Weakness in the arm or leg.
- Pain, aching, or heaviness in the arm or leg.
- Redness, swelling, or signs of infection.
Considerations for Teaching Patients Prevention and Control of
- Keep the arm or leg raised above the level of the heart,
when possible. Avoid making rapid circles with the arm or leg to keep
blood from collecting in the lower part of the limb.
- Clean the skin of the arm or leg daily and moisten with lotion.
- Avoid injury and infection of the arm or leg:
- Use an electric razor for shaving.
- Wear gardening and cooking gloves.
- Use thimbles for sewing.
- Take care of fingernails; do not cut cuticles.
- Keep the feet covered when outdoors.
- Keep the feet clean and dry; wear cotton socks.
- Cut toenails straight across; see a podiatrist as needed to prevent ingrown nails
- Either arms or legs:
- Suntan gradually; use sunscreen.
- Clean cuts with soap and water, then use antibacterial ointment.
- Use gauze wrapping instead of tape; do not wrap so tight that circulation is
- 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 or heating pads.
- Do not overwork the affected arm or leg.
- 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.
- Watch for signs of infection, such as redness, pain, heat,
swelling, and fever. Call the doctor immediately if any of these signs
- Do prescribed exercises regularly as instructed by the doctor or therapist.
- Keep regular follow-up appointments with the doctor.
- Check all areas of the arms and legs every day for signs of problems:
- Measure around the arm or leg at regular time
periods as suggested by the doctor or therapist.
- Measure the arm or leg at the same two places each time.
- Tell the doctor if the limb suddenly gets larger.
- The ability to feel touch, temperature, or pain in the affected
arm or leg may be lessened. Use the unaffected limb to test temperatures
for bath water or cooking.
Lymphedema is treated by physical methods
and with medication. Physical methods include supporting the arm or leg
in a raised position; manual lymphatic drainage (a specialized form of
very light massage that helps to move fluid from the end of the limb toward
the trunk of the body); wearing custom-fitted clothes that apply controlled
pressure around the affected limb; and cleaning the skin carefully to prevent
infection. Lymphedema may be treated by combining several therapies. This
is known as complex physical therapy (or complex decongestive therapy),
which consists of manual lymphedema treatment, compression wrapping, individualized
exercises, and skin care, followed by a maintenance program. Complex physical
therapy must be performed by a professional trained in the techniques.
Surgery for treating lymphedema usually
results in complications and is seldom recommended for cancer patients.
When pressure garments are used, they should
cover the entire area of edema. For example, a stocking that reaches only
to the knee tends to become tight and block the lymphatic vessels and veins
if there is edema in the thigh. Pumps connected to cuffs that wrap around
the arm or leg and apply pressure on and off may be helpful; however, some
physicians and therapists feel these pumps are not effective and may make
the edema worse. The cuff is inflated and deflated according to a controlled
time cycle. This pumping action is believed to increase the movement of
fluid in the veins and lymphatic vessels and keeps fluid from collecting
in the arm or leg. Compression pumps should be used only under the supervision
of a trained health care professional because high external pressure can
damage the lymphatic vessels near the skin surface.
Antibiotics may be used to treat and prevent
infections. Other types of drugs such as diuretics or anticoagulants (blood
thinners) are generally not helpful, and may make the problem worse.
Finding the exact cause of the swelling
and treating it correctly is important. Edema often leads to infection,
which then increases fluid and protein deposits in the tissues. If an infection
is diagnosed, appropriate antibiotics should be given. Blood clots should
be ruled out because massage and other therapy techniques to encourage
drainage may cause the clots to move through the bloodstream and cause
more serious heart or lung problems. If blood clots are found, they should
be treated with anticoagulants.
Coumarin is a dietary supplement that has
been studied as a treatment for lymphedema. In the United States, dietary
supplements are regulated as foods not drugs. Supplements are not required
to be approved by the Food and Drug Administration (FDA) before being put
on the market. Because there are no standards for manufacturing consistency,
dose, or purity, there may be considerable difference from one lot of dietary
supplements to the next.
Coumarin was once used in some foods and
medications in the United States. It was found to cause liver damage, and
its use in foods and medications in the United States has been banned since
the 1950s. Coumarin is available in several countries, but has not been
approved for use in the United States or Canada.
The nutritional status of the patient should
be evaluated and appropriate dietary recommendations should be made. Blood
protein levels and weight should be monitored regularly and patients should
be encouraged to eat protein-rich foods.
Patients with lymphedema may experience
pain caused by the swelling and pressure on nerves; loss of muscle tissue
and function; or scar tissue causing shortening of muscles and less movement
at joints. Pain may be treated with medications, relaxation techniques,
and/or transcutaneous electrical nerve stimulation (TENS), however, the
most successful treatment is to decrease the lymphedema.
The edema can make tissues less able to
take in nutrients and more likely to be damaged if the affected limb is
not moved for long periods. Therefore, patients with lymphedema should
be monitored for areas of skin breakdown, especially over areas where there
is very little tissue between the skin and bone (i.e., hips, knees, and
Bladder emptying problems can develop from
lymphedema in the pelvic or groin areas. Patients with lymphedema who are
also taking narcotic medications may develop bowel problems. Bowel and
bladder status should be monitored regularly for any signs of urine retention
Because lymphedema is disfiguring and sometimes
painful and disabling, it can create mental, physical, and sexual problems.
Several studies have noted that 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 treatment that is often painful, difficult,
Coping with lymphedema in the arm 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, and emotional support.
In addition to the complications associated
with chronic lymphedema noted in previous sections, 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 a little over 1 year.
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