Lymphedema pathophysiology

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Editors-in-Chief: Benoit Blondeau, M.D. [1]; C. Michael Gibson, M.S., M.D. [2]

Overview

Pathophysiology

Lymphedema may be due to:

• Obliterative disease of lymph nodes
• Hyperplastic disease of lymph nodes

Lymphedema (also see Elephantiasis) may be inherited (primary) or caused by injury to the lymphatic vessels (secondary). It is most frequently seen after lymph node dissection, surgery and/or radiation therapy, in which damage to the lymphatic system is caused during the treatment of cancer, most notably breast cancer. In many cancer patients this condition does not develop until months or even years after their therapy has concluded. Lymphedema may also be associated with accidents or certain diseases or problems that may inhibit the lymphatic system from functioning properly. In tropical areas of the world, a common cause of secondary lymphedema is filariasis, a parasitic infection.

While the exact cause of primary lymphedema is still unknown, it generally occurs due to poorly-developed or missing lymph nodes and/or channels in the body. Lymphedema may be present at birth, develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). Some cases of lymphedema may be associated with other vascular abnormalities. In the lower extremity it will be unilateral or bilateral. If it is bilateral, one leg may be worse than the other.

Lymphedema affects both men and women. In women, it is most prevalent in the upper limbs after breast cancer surgery and lymph node dissection, occurring in the arm on the side of the body in which the surgery is performed. It may also occur in the lower limbs or groin after surgery for colon, ovarian or uterine cancer in which removal of lymph nodes is required. In men, lower-limb primary lymphedema is most common, occurring in one or both legs. Surgery and/or treatment for prostate, colon and testicular cancers may result in secondary lymphedema, particularly where lymph nodes have been removed or damaged.

The onset of secondary lymphedema in patients who have had cancer surgery has also been linked to aircraft flight (likely due to decreased cabin pressure). For breast cancer survivors, wearing a prescribed and properly-fitted low-compression sleeve and gauntlet may help decrease swelling during flight.

Some cases of lower-limb lymphedema have been associated with the use of Tamoxifen, due to the blood clots and deep vein thrombosis (DVT) that can be caused by this medication.

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