Kaposi’s Sarcoma and Lymphedema
A Review of the Literature When Kaposi’s Sarcoma burst into the news some 20 years ago, it quickly became known as a cancer associated with HIV/AIDS. Interestingly, KS has shown that in and of itself, the cancer causes lymphedema by damaging the lymphatic system. Since that time, however, there have been two more groups of patients who have been clinically shown to be at risk. The first groups is that of organ transplants. Indeed, a study done by the Mayo Clinic in Rochester, Minnesota demonstrated that the risk of contracting Kaposi’s Sarcoma is 500 times greater in the organ transplant population then that of the general population. The reason for this is simple. Organ transplant patients must take immunosuppressive drugs to prevent organ rejection. The next group that is now showing a proclivity towards Kaposi’s Sarcoma is comprised of long term lymphedema patients. This group includes both primary and secondary lymphedema. A lymphedematous limb has long been understood to become immunodeficient and there is even conjecture from early studies that indeed even those with secondary lymphedema may already have an “at risk” lymph system, thereby possibly causing a lowered immune response throughout their body.
Arm and Leg Swelling After Cancer
With the advent of better and more effective cancer treatments, the survival rate for all cancers has risen dramatically. With this progress, a new and often misunderstood and misdiagnosed complication has arisen.
Many cancer survivors , having overcome cancer, find themselves with sudden and often unexplained swelling, usually of the arms or of the legs.
This swelling occurs because of one of several factors.
First, the swelling begins after lymph nodes have been removed for cancer biopsies.
Second, the swelling may start as a result of radiation damage to either the lymph nodes and/or the lymph system.
Due to either the removal of lymph nodes or damage to the lymph system, your body is no longer able to rid itself of excess fluids. The fluids collect in the limbs effected and swelling beings.
This swelling is called lymphedema. The swelling that occurs is permanent, and while it is not curable it is treatable.
Permanent Leg Swelling
****In the situation of any permanent leg swelling whether the cause is known or unknown, the diagnoses of lymphedema must be considered****
There are several groups of people who experience leg swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.
This group includes those who have had the injuries, infections, insect bites, trauma to the leg, surgeries or reaction to a medication. When this swelling does not go away, and becomes permanent it is called secondary lymphedema.
Another extremely large group that experiences permanent leg swelling are cancer patients, people who are morbidly obese, or those with the condition called lepedema. What causes the swelling to remain permanent is that the lymph system has been so damaged that it can no longer operate normally in removing the body's waste fluid. In cancer patients this is the result of either removal of the lymph nodes for cancer biopsy, radiation damage to the lymph system, or damage from tumor/cancer surgeries. This is also referred to as secondary lymphedema.
Group three consists of people who have leg swelling from seemingly unknown reasons. There may be no injury, no cancer, no trauma, but for some reason the leg simply is swollen all the time.
The swelling may start at birth, it may begin at puberty, or may begin in the 3rd, 4th or even 5th decade of life or sometimes later. This type of leg swelling is called primary lymphedema. It can be caused by a genetic defect, malformation or damage to the lymph system while in the womb or at birth or be part of another birth condition that also effects the lymph system. This is an extremely serious medical condition that must be diagnosed early, and treated quickly so as to avoid painful, debilitating and even life threatening complications. Treatment should NOT include the use of diuretics.
What is Lymphedema?
Lymphedema is defined simply as an accumulation of excessive protein rich fluid in the tissues of the leg. The accumulation of fluid causes the permanent swelling caused by a defective lymph system.
A conservative estimate is that there may be 1-2 million people in the United States with some form of primary lymphedema and two to three million with secondary lymphedema.
What are the symptoms of Lymphedema?
If you are an at risk person for leg lymphedema there are early warning signs you should be aware of. If you experience any or several of these symptoms, you should immediately make your physician aware of them.
1.) Unexplained aching, hurting or pain in the leg.
2.) Experiencing "fleeting lymphedema." This is where the limb may swell, even slightly, then return to normal. This may be a precursor to full blown leg lymphedema.
3.) Localized swelling of any area. Sometimes lymphedema may start as swelling in one area, for example the foot, or between the ankle and knee. This is an indication of early lymphatic malfunction.
4.) Any arm inflammation, redness or infection.
5.) You may experience a feeling of tightness, heaviness or weakness of the leg.
How is Lymphedema Treated?
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. It is a form of massage therapy where the leg is very gently massaged to actually move the fluid out of the leg and into an area where the lymph system still functions normally. With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down and/or is taught to use compression wraps to maintain the leg size.
What are some of the complications of lymphedema?
1. Infections such as cellulitis, lymphangitis, erysipelas. This is due not only to the large accumulation of fluid, but it is well documented that lymphodemous limbs are localized immuno-deficient.
2. Draining wounds that leak lymphorrea which is very caustic to surrounding skin tissue and acts as a port of entry for infections.
3. Increased pain as a result of the compression of nerves usually caused by the development of fibrosis and increased build up of fluids.
4. Loss of Function due to the swelling and limb changes.
5. Depression - Psychological coping as a result of the disfigurement and debilitating effect of lymphedema.
6. Deep venous thrombosis again as a result of the pressure of the swelling and fibrosis against the vascular system. Also, can happen as a result of cellulitis, lymphangitis and infections.
7. Sepsis, Gangrene are possibilities as a result of the infections.
8. Possible amputation of the limb.
9. Pleural effusions may result if the lymphatics in the abdomen or chest are to overwhelmed to clear the lung cavity of fluids.
10. Skin complications such as splitting, plaques, susceptibility to fungus and bacterial infections.
11. Chronic localized inflammations.
Can lymphedema be cured?
No, at the present time there is no cure for lymphedema. But it can be treated and managed and most of the complications can be avoided. Life with lymphedema can still be active and full, with proper treatment, patient education, and patient life style adaptation.