What is Lymphoedema
Lymphoedema (lymphedema) occurs when the lymphatic system does not work properly. This results in the long-term swelling of part or parts of the body. The lymphatic system is similar to the blood system and has just as many vessels - but they contain lymph, which is clear and so cannot be seen (unless a suitable dye is injected). The lymphatics differ from the blood system in that the blood continually circulates through each part of the body while the lymph just drains from each part.
Lymphatics drain away the excess protein and water which continually escape from the blood in small amounts, plus some substances made in the tissues, and any foreign substances which enter them. Lymphatics start, in almost every tissue, as many tiny vessels which gradually join together into bigger ones.
Lymph is pumped into and along these vessels by the movements of adjacent muscles and by the contractions of the walls of the larger lymphatics. This pumping is aided by many valves inside the vessels. Finally the lymphatic system empties into the blood (largely in the lower neck). On its way along the lymphatics, the lymph is filtered in the lymph nodes (lymph glands). These remove foreign matter (e.g. bacteria) and to start any necessary immune reactions.
The lymphatics help to remove the excess fluid and protein which enters the tissues from damaged blood vessels, in any inflammation (e.g. after a burn, or other injury). If they cannot remove it all, the part swells (oedema, edema). However this swelling is usually only temporary, because the tissues heal and the blood vessels no longer leak excessively.
In an acute injury, e.g. a sprained ankle, the lymphatics are essentially normal. Although there is initial swelling, this is gradually removed over days to weeks. The overload is only temporary although, depending on the severity of the injury, some fibrosis will occur. This may remain for months or even permanently.
However if the lymphatic system is damaged or blocked, protein continues to enter the tissues from the blood capillaries in the normal way, and a build-up occurs in the tissues the lymphatics should be draining. The accumulation of protein in the tissues causes excess fluid to enter them and the tissues to swell. The swelling decreases the oxygenation of the tissues, interferes with their normal functioning, and makes them heal more slowly than normal.
To some extent, the protein is also removed by some of the cells in the tissues (e.g. the macrophages). These assist the lymphatic system and can partly take over its role if it is blocked. However in lymphoedema, the chronic excess protein causes these cells also to cease to function.
The excess protein also acts as a stimulus for chronic inflammation. One of the results of this is the formation of much excess fibrous tissue. The chronic inflammation causes more blood capillaries to form and to be dilated. This makes the limb feel hot.
This heat, combined with the stagnant protein provides a perfect site for bacterial growth (Secondary Acute Inflammation, S.A.I.). The patient may be very ill, with constant infections, and need hospitalisation. Fungal infections are also very frequent, and are often difficult to clear up. These infections, themselves, place greater loads on the lymphatics, and so worsen the lymphoedema. Any infection or other inflammation (e.g. after injury, sunburn, etc.) makes the lymphoedema worse.
If the swelling is rapid (e.g. after an operation), it can cause great pain. This is because the tissues are being torn apart. Adjacent areas, which are receiving excess lymph diverted from the blocked region, also often ache (e.g. the shoulder adjacent to a lymphoedematous arm). If swelling is slow (e.g. primary lymphoedema), there may be no pain at all, except during bouts of infection.
Lymphoedema is usually considered only to occur in the arm or leg, but any organ or region may suffer from it: the genitals, the gut, the lungs, the liver as a consequence of chronic hepatitis, etc.
Lymphoedema has a number of other effects: it decreases mobility, causes embarrassment, can lead to depression, and causes a general worsening of the patient's life and health. If the lymphoedema is severe, especially if more than one limb is involved, the patient is excessively heavy. This may be confused with obesity, but dieting is useless if it is just lymphoedema; however if there is obesity, dieting is recommended. Even if only one limb is affected, the added weight can cause clumsiness and this makes the limb more liable to injury and consequent bacterial infection.
Lymphatic drainage from the legs is more difficult than from the arms, walking is more difficult and the excess weight discourages this. So lymphoedema of the legs is often worse than that of the arms.
When lymphoedema first occurs, it will pit if pressed with a thumb. It gradually becomes larger and harder; it no longer pits. If lymphoedema lasts for some years, the swelling gets worse, and skin changes occur. There is loss of hair and alterations to the nails. The skin may get very thick, with huge folds and warts - elephantiasis. A few patients with long-term lymphoedema develop an angiosarcoma (Stewart-Treves Syndrome). Any lymphoedema left untreated will gradually progress along this route.
Reference: Lymphoedema Association of Australia