Lymphedema is a condition where body fluids, typically carried though a fine network of vessels known as lymph vessels, become trapped in the tissue due to obstruction, absence or scarring of the lymph channels or some other condition, resulting in swelling and deformity of the soft tissues and skin. Lymphedema conditions can be mild or severe and can be caused by a variety of conditions and can affect literally any part of the body, although upper and lower extremities are the most commonly affected areas.

Lymph fluid serves several functions.  First, lymphatic fluid is rich in white blood cells which help to attack, neutralize or introduce harmful pathogens to other immune system defenses. Lymphatic fluid also serves as an important connector of white blood cell growth and maturation centers known as lymph nodes. Lymph nodes contain large concentrations of immune system defenses and help to isolate and control the spread of infection. Individuals who have obstructed lymph flow systems or who have had lymph nodes removed, are at risk for stasis or low or no flow lymph states and are also susceptible to infection as they lack either the nodes or the pathways designed to fight infection. Together lymph fluid, lymph channels and lymph nodes comprise the lymphatic system.

In an ideally functioning body that does not have lymphatic injury, lymphatic fluid circulates aided by manual compression that occurs when muscles and tissue compress the vessels and low pressure flow gradients. In the compromised lymphatic system however, these flow gradients are usually absent or aberrant and lymphatic flow relies almost completely on manual compression. Often time, exaggerated compression forces must be achieved in order to direct lymph back toward the area where it is recycled and where it enters the cleansing cycle.

Once the lymph system is injured, a variety of insults may exacerbate or lead to complications such as infection, swelling, skin breakdown, and compartment syndrome. For this reason, persons with lymphedema should generally minimize trauma such as the application of tourniquets or blunt force trauma and in the affected limb, intravenous access, needle sticks, intramuscular or subcutaneous injections and/or PICC lines should not be attempted.   Such interventions may trigger a cascade of injury resulting in infection , tissue breakdown, increased swelling and edema which can lead to compartment syndrome and/or auto-necrosis, and in extreme cases cause the need for amputation and can even cause death.

A variety of conditions lead to lymphedema.  These conditions which are are grouped by the mechanism which caused them, include  primary and secondary lymphedema.

Primary Lymphedema:  Primary lymphedema includes a variety of congenital, genetic or acquired conditions which result in varying degrees of lymphedema. The following list includes some of the conditions which have been associated with causing primary lymphedema:

1. Milroy’s Disease (congenital lymphedema)

2. Klippel-Trenaunay-Weber Syndrome

3.  Meige’s Disease (lymphedema praecox)

4. Late onset lymphedema (lymphedema tardae)

5. Hair Tourniquet Syndrome

Secondary  Lymphedema:  Secondary  lymphedema includes a variety of conditions generally caused by surgery or injury, which result in varying degrees of lyphedema. The following list includes some of the conditions which have been associated with causing secondary lymphedema:

1. Surgical removal or dissection of lymph nodes.  Surgical removal of the lymph nodes is a common intervention in the surgical treatment of breast cancer and head and neck cancers. Biopsy and surgical removal of a single suspicious lymph node infrequently can also lead to lymphedema. Mastectomy is the surgical intervention most commonly associated with secondary lymphedema.

2. Radiation therapy

3. Drugs.  Tamoxifen has been implicated with causing lymphedema and there are a variety of agents which can exacerbate fluid accumulation.  These drugs include: certain dihydropyridine class calcium channel blockers such as amlodipine,  felodipine, nicardipine and nifedipine certain anti-glycemic agents such as those used to treat diabetes including agents such as glipizide, glyburide, and thioglitazones such as rosiglitazone.

4. Malignancy.  Malignant conditions can lead to blockage or destruction of the lymph nodes or lymph channels which can in turn lead to lymphedema. In breast cancer, a noted characteristic of late stage breast cancer was found when the breast becomes very swollen, hard and discolored ad takes on an orange peel like quality refered to in the medical literature as peau’ de orange.  Other malignancies such as Hodgkin’s lymphoma, can also cause lymphedema

5. Filariasis

6. Bacterial, viral and fungal infections:

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Cancerquest Video: Lymphedema