LYMPHEDEMA PRIMARY AND SECONDARY

Primary Lymphedema

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THE LYMPH SYSTEM

The lymph system is one of your bodies circulatory systems. It is composed of lymph vessels, lymph nodes and organs such as the bone marrow, spleen, thymus and is also believed to include tonsils. Is function includes the absorption and elimination of excess fluids, it assists in the absorption of fat and very important is critical to our bodies ability to fight infections and is critical in the immune system functions. When this system is not formed correctly, is damaged through injury or infection or is removed (nodes) then lymphedema is a very real possibility.

LYMPHEDEMA

As mentioned previously lymphedema is a condition that occurs from a damaged or dysfunctional lymphatic system. There are two different types of lymphedema.

PRIMARY LYMPHEDEMA

PRIMARY LYMPHEDEMA can be hereditary. Milroy's Syndrome or Desease will generally express itself at birth or in the very early years. Meige Lymphedema, also known as lymphedema praecox generally begins sometime during puberty. Lymphedema tarda begins in or around middle age. Lymphedema that has not expressed itself in an active condition is referred to a latent lymphedema. Primary lymphedema can also be congenital. This means some either in utero or during birth caused lymphatic damage.

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TYPES OF PRIMARY LYMPHEDEMA

MILROY'S SYNDROME

Related Terms: Nonne-Milroy lymphedema, Milroy's Disease, Primary congenital hereditary lymphedema, hereditary lymphedema I, Nonne-Milroy-Meige disease

Milroy's Syndrome is an old term used to describe hereditary congenital lymphedema. It is a congenital familial primary lymphedema which results from vertical autosomal inheritance of a single gene. The gene has been identified as VEGFR3. The condition usually presents itself at birth with the swelling of one or even both legs.

If the condition is unilateral (single leg), the other leg may continue in the latency stage for years before expressing itself. The same is indicated for arm lymphedema.

It is the rarest of the inherited lymphedema, accounting for approximately 2% of hereditary lymphedemas.

Hereditary lymphedema was first described by Nonne in 1891, however in 1892 Dr. William F. Milroy described a missionary who had returned from work in India who had swollen legs his entire life. His mother likewise was afflicted with the same condition. Milroy had also, previously studied the 250 year history of a family and had been able to identify 22 persons with this condition through 6 generations. He was also able to pinpoint when the condition entered the family through a marriage in 1768.

Diagnosis

Basic diagnosis can be made by the fact that swelling (generally of the legs) presents at birth and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.

Etiology

The cause of Milroy's Syndrome is a break in the VEGFR3 gene.

Complications

The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.

Treatment

Decongestive therapy is the most widely accepted form of treatment. There is no cure for Milroy's but the condition can be managed by early diagnosis and treatment.

Prognosis

Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.

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MEIGE LYMPHEDEMA SYNDROME

See also: Nonne-Milroy-Meige Syndrome, Meige's lymphedema, Hereditary lymphedema II, familial lymphedema praecox, Lymphedema praecox meige

Form of primary hereditary lymphedema that starts at or around the time of puberty. The affected limbs are generally the legs.

Named after French physician Dr. Henri Meige who first described hereditary lymphedema in 1891. This form of lymphedema which usually presents itself at or during puberty is the most common of the hereditary lymphedemas, account for 65-80% of all diagnosed cases.

Meige-Type Lymphedema

Also known as Lymphedema II, this syndrome is similar to Lymphedema I but the onset of peripheral edema occurs during the second to the fifth decades. The legs are the most commonly involved, and lymphangiography reveals hypoplasia of peripheral lymphatics with dilation of lymphatic trunks.

Diagnosis

Basic diagnosis can be made by the fact that swelling (generally of the legs) presents during puberty and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.

Etiology

The cause of Lymphedema Praecox is a break in the FOXC2 gene.

Complications

Other Indications

Related conditions may also include yellow nail syndrome, pulmonary hypertension, cerebrovascular malformations and distichiasis

Treatment

Decongestive therapy is the most widely accepted form of treatment. There is no cure for Lymphedema praecox but the condition can be managed by early diagnosis and treatment.

Prognosis

Long term prognosis is excellent if the condition is identified early and treatment begins so after the diagnosis is made.

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LYMPHEDEMA TARDA

Form of primary hereditary lymphedema that expresses itself during middle age (generally onset 35+ years). Swelling generally occurs in the legs and may involve either one or both limbs. There is a higher incidence of lymphedema tarda among females than males.

This form of inherited lymphedema accounts for approximately 10% of those with primary lymphedema.

Diagnosis

Basic diagnosis can be made by the fact that swelling (generally of the legs) unexpectedly and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.

Etiology

The cause of lymphedema tarda is a break in the FOXC2 gene.

Complications

Treatment

Decongestive therapy is the most widely accepted form of treatment. There is no cure for lymphedema tarda but the condition can be managed by early diagnosis and treatment.

Prognosis

Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.

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SECONDARY LYMPHEDEMA

SECONDARY LYMPHEDEMA is generally caused by an obstruction, damage to or injury to the lymph system that leads to an interruption of the normal lymphatic flow.

CAUSES

PRIMARY LYMPHEDEMA - The cause of hereditary primary lymphedema has been isolated to a malformation or break in two known genes. These are the FOXC2 and VEGFC genes. There is a suspected third gene, but as yet, it has not been identified. Causes of congenital primary lymphedema can be a developmental disorder of the lymphatics, in utero infection or injury and/or delivery difficulties.

SECONDARY LYMPHEDEMA - The causes of secondary lymphedema are multiple. Infections from insect bites, serious wounds, or burns can cause lymphedema when they damage or destroy lymphatics as kind any type of serious injury, radiation for cancer treatments is also a cause. Outside the tropics the number one cause of secondary lymphedema is the removal of lymph nodes for cancer biopsies. Hopefully, with the improved techniques of small needle biopsies, radiological diagnostic improvements and site specific node biopsies we will sees a marked decrease in this type of lymphedema.

In the tropical climates the most common cause of lymphedma is infection from filarial worms. Generally, resulting from mosquito bites. This parasite then grows eventually blocking and destroying the lymphatic system.

TREATMENT OPTIONS

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.

Other treatments include the use of compression pumps, surgery, and newer approaches such as the use of lasers, liposuction, wholistic therapies and even acupuncture.

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HERE IS AN EXCELLENT ARTICLE FROM THE NATIONAL CANCER INSTITUTE
INFORMATION FROM PDQCancerMail from the National Cancer Institute Information from PDQ for Patients

Lymphedema

** INTRODUCTION ** 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 trials, is available from the National Cancer Institute. Lymphedema is the buildup of lymph (a fluid that helps fight infection and disease) in the fatty tissues just under the skin. The buildup of lymph causes swelling in specific areas of the body, usually an arm or leg, with an abnormally high amount of tissue proteins, chronic inflammation, and thickening and scarring of tissue under the skin. Lymphedema is a common complication of cancer and cancer treatment and can result in long-term physical, psychological, and social issues for patients.

**OVERVIEW ** 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.

Acute versus gradual-onset 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 versus chronic lymphedema

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 - inflammation - 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.

Risk factors

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 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 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, 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. - An inadequate diet or those who are overweight. These conditions may delay recovery and increase the risk for lymphedema.
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 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.

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: - 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 Table 1 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 experience lymphedema. 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.

MedHelp

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ADDITIONAL INFO ON LYMPHEDEMA

Highest Recommendation to Read

I have corresponded with Dr. Gogia as he is part of the Lymphology Association of India. His book is in my opinion the finest single document on lymphedema available on the internet. I would highly suggest reading it.

Book on Lymphoedema/Lymphedema

Dr. S. B. Gogia

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HGF and MET mutations in primary and secondary lymphedema.

http://www.liebertonline.com/doi/abs/10.1089/lrb.2008.1524

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Secondary Lymphedema References

http://www.hgen.pitt.edu/projects/lymph/references.php#sec

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Excerpt from Milroy Disease

http://www.emedicine.com/med/byname/milroy-disease.htm

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Lymphedema

http://www.meb.uni-bonn.de/cancer.gov/CDR0000062735.html

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Lymphedema

http://www.cancer.net/portal/site/patient/gsasearch?q=lymphedema&x=0&y=0

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Lymphedema

Author: Don R Revis, Jr, MD, Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine

www.emedicine.com/med/topic2722.htm

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Lymphedema

http://www.medifocus.com/lymphedema.asp

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Management of Extremity Lymphedema

http://www.moffitt.usf.edu/pubs/ccj/v2n5/article6.html

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Lymphedema - MedicineNet

http://www.medterms.com/script/main/Art.asp?ArticleKey=4219

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Supportive care statement for Health professionals

Lymphedema

http://www.meb.uni-bonn.de/cancer.gov/CDR0000062735.html

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Milroy Disease

Last Updated: January 20, 2003

Synonyms and related keywords: congenital lymphedema, lymphedema congenita, noninfectious hereditary elephantiasis, autosomal dominant lymphedema, lymphatic obstruction, fibrosis, cellulitis, Meige disease, lymphedema tarda, lymphedema praecox

Author: Raphael J Kiel, MD, Associate Program Director, Head of Infectious Disease Section, Associate Professor of Internal Medicine, Department of Internal Medicine, Oakwood Hospital, Wayne State University School of Medicine

http://www.emedicine.com/med/topic1482.htm

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Secondary Lymphedema: Non-Cancer Related

BONNIE B. LASINSKI, MA, PT, CI, CLT-LANA

http://www.lymphedema-therapy.com/03paper.htm

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Join us as we work for lymphedema patients everywehere:

Advocates for Lymphedema

Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.

http://health.groups.yahoo.com/group/AdvocatesforLymphedema/

Subscribe:

AdvocatesforLymphedema-subscribe@yahoogroups.com

Pat O'Connor

Lymphedema People / Advocates for Lymphedema

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For information about Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema\

For Information about Lymphedema Complications

http://www.lymphedemapeople.com/wiki/doku.php?id=complications_of_lymphedema

For Lymphedema Personal Stories

http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=3

For information about How to Treat a Lymphedema Wound

http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound

For information about Lymphedema Treatment

http://www.lymphedemapeople.com/wiki/doku.php?id=treatment

For information about Exercises for Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema

For information on Infections Associated with Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema

For information on Lymphedema in Children

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children

Lymphedema Glossary

http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing

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Lymphedema People - Support Groups

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Children with Lymphedema

The time has come for families, parents, caregivers to have a support group of their own. Support group for parents, families and caregivers of chilren with lymphedema. Sharing information on coping, diagnosis, treatment and prognosis. Sponsored by Lymphedema People.

http://health.groups.yahoo.com/group/childrenwithlymphedema/

Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com

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Lipedema Lipodema Lipoedema

No matter how you spell it, this is another very little understood and totally frustrating conditions out there. This will be a support group for those suffering with lipedema/lipodema. A place for information, sharing experiences, exploring treatment options and coping.

Come join, be a part of the family!

http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515

Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com

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MEN WITH LYMPHEDEMA

If you are a man with lymphedema; a man with a loved one with lymphedema who you are trying to help and understand come join us and discover what it is to be the master instead of the sufferer of lymphedema.

http://health.groups.yahoo.com/group/menwithlymphedema/

Subscribe: menwithlymphedema-subscribe@yahoogroups.com

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All About Lymphangiectasia

Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.

http://health.groups.yahoo.com/group/allaboutlymphangiectasia/

Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com

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Lymphatic Disorders Support Group @ Yahoo Groups

While we have a number of support groups for lymphedema... there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.

DISCRIPTION

Information and support for rare and unusual disorders affecting the lymph system. Includes lymphangiomas, lymphatic malformations, telangiectasia, hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.

http://health.groups.yahoo.com/group/lymphaticdisorders/

Subscribe: lymphaticdisorders-subscribe@yahoogroups.com.

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Lymphedema People New Wiki Pages

Have you seen our new “Wiki” pages yet? Listed below are just a sample of the more than 140 pages now listed in our Wiki section. We are also working on hundred more. Come and take a stroll!

Lymphedema Glossary

http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing

Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema

Arm Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=arm_lymphedema

Leg Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=leg_lymphedema

Acute Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=acute_lymphedema

The Lymphedema Diet

http://www.lymphedemapeople.com/wiki/doku.php?id=the_lymphedema_diet