Oral HIV-Associated Kaposi Sarcoma: A Clinical Study from the Ga-Rankuwa Area, South Africa.

2012

Khammissa RA, Pantanowitz L, Feller L.

Source

Department of Periodontology and Oral Medicine, University of Limpopo, Medunsa Campus, Pretoria 0204, South Africa.

Abstract

Background. Kaposi sarcoma (KS) is one of the most common neoplasms diagnosed in HIV-seropositive subjects. Oral involvement is frequent and is associated with a poor prognosis. The aim of this study was to characterize the features of oral HIV-KS in patients from Ga-Rankuwa, South Africa. 

Methods. All cases with confirmed oral HIV-KS treated at the oral medicine clinic in Ga-Rankuwa from 2004 to 2010 were included in this retrospective study. Differences between males and females with oral HIV-KS in relation to HIV infection status, to oral KS presentation and to survival rates were statistically analysed. 

Results. Twenty (54%) of the 37 patients in the study were females and 17 (46%) were males. In 21 patients (57%), the initial presentation of HIV-KS was in the mouth. Other than the fact that females presented with larger (≥10 mm) oral KS lesions (P = 0.0004), there were no statistically significant gender differences. Significantly more patients presented with multiple oral HIV-KS lesions than with single lesions (P = 0.0003). Nine patients (24%) developed concomitant facial lymphoedema, and these patients had a significantly lower CD4+ T-cell count (28 cells/mm(3)) compared to the rest of the group (130 cells/mm(3)) (P = 0.01). The average CD4+ T-cell count of the patients who died (64 cells/mm(3)) was significantly lower (P = 0.0004), there were no statistically significant gender differences. Significantly more patients presented with multiple oral HIV-KS lesions than with single lesions (P = 0.016) at the time of oral-KS presentation than of those who survived (166  cells/mm(3)). 

CONCLUSIONS: In Ga-Rankuwa, South Africa where HIV-KS is prevalent, oral KS affects similarly males and females. A low CD4+ T-cell count at the time of oral HIV-KS diagnosis and the development of facial lymphoedema during the course of HIV-KS disease portends a poor prognosis.

NIH

Lymphangioma-Like Kaposi Sarcoma.

August 2012

We report on a recent lesion in a 75-year-old woman who

had been diagnosed with classic Kaposi sarcoma (KS) 8 years earlier, at which time she presented with plaques on the right thigh and right forearm (Fig. 1A) and no metastasis was detected. Findings from the initial biopsy of the forearm lesions were compatible with nodular KS.

During the 8-year follow-up, the patient developed 7 recurrent skin tumors on the forearm and legs. Each tumor had the same clinical appearance in the form of plaques (Fig. 1B), and no systemic involvement was detected at any time. The lesions were treated with liposomal doxorubicin. Biopsies were taken from the forearm at each recurrence and histology continued to show findings consistent with nodular KS mixed with lymphangioma-like areas. 

These areas were comprised of irregularly-dilated ectatic vascular spaces in the reticular dermis that were lined by moderately atypical endothelial cells. These spaces were greater in number and size than normal lymphatic vessels (Fig. 2). Immunohistochemistry showed strong CD34 positivity. Endothelial cells lining the lymphangioma-like areas of the tumor were

also positive on staining with human herpes virus type 8

(HHV-8) antibody (Fig. 2C) and the lymphatic endothelial

marker podoplanin (D2-40) (Fig. 2D). Based on these findings, the patient was diagnosed with lymphangioma-like KS.

Seven months after completing the most recent cycle of

chemotherapy with doxorubicin, the patient presented with a serious recurrence consisting of localized, multinodular, ulcerated lesions on both legs but more severe on the right thigh; severe lymphedema was also noted (Fig. 1C). She was given radiotherapy, second-line chemotherapy with paclitaxel (taxol), and thorough local treatments. At the time of writing the lesions remained ulcerated and had not changed in size. The patient attended scheduled follow-up visits and did not show signs of systemic spread of disease.

Full Text Article with Diagnostic Images

Elsevier

Understanding the Lymph System

Understanding the Lymph System

I thought it would be helpful for readers to understand the lymph system, the anatomy, what it does, and how it helps with immunity.

Listed below are information pages that should be quite helpful and each page has many additional links for more a more in depth study.

Anatomy of the Lymph System

Lymphatic System Functions

Lymphatic System and Immunity

Pathology of the Lymph Nodes and Lymphoma

Lymph Nodes

Lymph Fluid

Arm and Leg Swelling After Kaposi's Sarcoma

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 begins.

This swelling is called lymphedema and it can effect either your leg or your arm. The swelling that occurs is permanent, and while it is not curable it is treatable.

Hopefully, in the future with radiological scans becoming more sensitive and with increased use of such techniques such as the small needle biopsy, we can put an end to this epidemic of secondary lymphedema from cancer biopsy.

Permanent Leg or Arm Swelling

In the situation of any permanent leg

There are several groups of people who experience leg or arm swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.

Group One

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.

Group Two

Another extremely large group that experiences permanent leg or arm swelling arecancer 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

Group three consists of people who have leg or arm 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 or arm 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 arm 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 arm

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 arm lymphedema.

3.) Localized swelling of any area. Sometimes lymphedema may start as swelling in one area, for example the hand, or between the elbow and hand. 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 arm.

The symptoms for leg lymphedema are very similar to ones for the arm.

HOW IS LYMPHEDEMA TREATED?

The preferred treatment today is decongestive therapy. The forms of therapy are complete decongestive therapy (CDT) or manual decongestive therapy (MLD), 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 immunocompromised.

2. Draining wounds that leak lymphorrhea 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.

For extensive information on lymphedema, please visit our home page:

Lymphedema People

Kaposi’s Sarcoma, Leg or Arm Swelling and Lymphedema

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.

Group One

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.

Group Two

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

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.