LYMPHOEDEMA FRAMEWORK

What Is Lymphedema?

The Lymphatic system

The lymphatics form part of your immune system, helping to deal with infection at a local level but just as importantly, they are responsible for cleansing your tissues and maintaining a balance of fluids in your body.
It can be likened to a waste disposal system, taking tissue fluid, bacteria, proteins and waste products away from the tissues around skin, fat, muscle and bone.
Once inside the lymphatic vessels (not visible just under the surface of the skin) the tissue fluid becomes known as ‘lymph’ and it is then transported in one direction, by increasingly larger and deeper lymphatic vessels. Movement of lymph depends on muscle movement (exercise) and the contraction of the deeper vessels themselves. Gentle massage known as Manual Lymphatic Drainage (MLD) or Self Lymphatic Drainage and deep breathing can also help to move the lymph more effectively.
At some point in its journey, lymph will pass through a lymph node, or gland. Clusters of these nodes are found in the neck, armpits and groins. It is here that the lymph is filtered and cleansed, so that the waste matter, bacteria and harmful cells can be identified and removed by the body’s defense system.
Having passed through these nodes, lymph finally drains back into the large veins of the body. From here it travels in the blood back to the heart and is eventually removed from the body as urine through the kidneys.

What is lymphedema?

Lymphedema may manifest as swelling of one or more limbs and may include the corresponding quadrant of the trunk. Swelling may also affect other areas, e.g. the head and neck, breast or genitalia. Lymphedema is the result of accumulation of fluid and other elements (e.g. protein) in the tissue spaces due to an imbalance between interstitial fluid production and transport (usually low output failure).
It arises from congenital malformation of the lymphatic system (Primary Lymphedema), or damage to lymphatic vessels and/or lymph nodes (secondary Lymphedema) A damaged lymphatic system can result from cancer treatment including the removal of lymph nodes, radiotherapy or can develop with the progression of malignant disease. The onset of swelling from lymphoedema may occur within months of the damage, or it may not appear until many years later.

Causes of primary lymphedema

Three gene mutations have been linked with primary lymphedema:

  • FOXC2 – lymphedema-distichiasis syndrome
  • VEGFR-3 – Milroy’s disease
  • SOX18 – hypotrichosis-lymphedematelangiectasia syndrome.

Causes of secondary Lymphedema

Secondary lymphoedema may also arise without a cancer diagnosis when one or more of the following conditions occur:

  • Trauma and soft tissue damage
  • Chronic Inflammation
  • Infection such as cellulitis – see Consensus Guideline: Management of cellulitis in lymphoedema
  • Filariasis – Lymphatic Filariasis is a major cause of lymphoedema in the sub -tropical areas of the world. Parasitic filarial worms are transmitted through mosquito bites. The parasites lodge in the lymphatic system causing destruction of the healthy vessels and nodes, resulting in lymphoedema.

Lymphoedema may also be of a mixed etiology. The lymphatic system is involved in the return of all interstitial fluid to the central blood circulation. Chronic lymphoedema may be caused by lymphatic failure associated with:

  • Venous disease
  • Immobility and dependency
  • Obesity
  • Lipoedema
  • Cancer or other chronic or palliative medical conditions

Early signs and symptoms of lymphedema

  • Clothing or jewelry, e.g. sleeve, shoe or ring, becoming tighter
  • Feeling of heaviness, tightness, fullness or stiffness
  • Aching
  • Observable swelling