Early /late primary lymphedema - Limfology

Early /late primary lymphedema

To understand the role of the lymphatic system in the body's defence mechanism and the diseases that affect it, we must imagine the lymph as a "carrier" for: the body's necessary nutrients, interstitial proteins, degradation products resulting from cellular metabolism (eliminates toxins) and not lastly, white blood cells or lymphocytes with an important role in immunity.

 The lymphatic system consists of about 100 lymph nodes located along the routes of the submandibular, laterocervical, axillary, inguinal lymph vessels, in the lower / upper limbs but also the internal organs (thoracic and abdominal).

What is lymphedema

Lymphedema is a condition of an inflammatory cause of the lymphatic circulatory system responsible for transporting compounds from the extracellular environment back into the blood circulation. The classification of lymphedema can be made according to the triggers of the disease as follows:

  • Rare primary lymphedema is mainly congenital and is due to abnormal development of the lymphatic system, and can be included in clinical syndromes such as: Milroy's disease (abnormal formation of lymph nodes during life) or Meige's disease (manifestations occur in people under 35 more often in women during puberty or in pregnancy);
  • Secondary lymphedema is due to an associated condition, surgery to damage the lymph nodes or vessels (such as axillary lymph node dissection in oncogenic mastectomies) or may be secondary to spontaneous trauma.

What causes lymphedema

Secondary lymphedema more frequently encountered in practice usually appears latent, this fact being explained by the high functional reserve capacity of the lymphatic system (in other words, the reserve volume that can be circulated is several times higher than the functional one), the main causes that determine the appearance of the disease being:

  • Surgery with lymph node involvement (axillary, submandibular, groin, mediastinal in thoracic or abdominal surgery);
  • Radiotherapy of the lymphatic system used in the treatment of various forms of neoplasm;
  • Parasites with nematodes such as Wuchereria bancrofti, Brugia malayi or Brugia timori (in Africa) transmitted by mosquito bites;
  • Tumours that may include and affect vessels or lymph nodes,in their evolution (local compression of bulky tumours);
  • Erysipelas, an infection of the superficial subcutaneous tissue due to group A hemolytic streptococcus or Staphylococcus aureus can affect the lymphatic circulation with the appearance of manifest lymphedema;
  • Trauma accompanied by bone fractures, deep burns affecting the lymphatic vessels;
  • Retroperitoneal fibrosis - known as Ormond syndrome (proliferation of fibrous tissue, most likely due to an immune response of the body with obstruction of lymphatic and blood vessels secondary to compression).

What are the specific symptoms?

The main manifestations of the disease are represented by:

  • Cutaneous edema in the territory served by the affected lymphatic vessels; the most common are lymphatic edema of the extremities (swelling of the affected limb sometimes including the fingers);
  • Erythema, reddening of the skin in the acute stage, with thickening of the skin in situations of chronic evolution of the disease;
  • Feeling of heaviness, discomfort, pressure and sometimes pain with functional impotence in severe cases of evolution;
  • In some situations, the clinical picture may be supplemented by paresthesias (tingling sensation, burning, stinging), chills, marked thirst and altered general condition.

Therapeutic approach to lymphedema

Lymphedema is a chronic disease, so therapeutic methods must be applied permanently due to the tendency to recover from edema. The treatment of the disease is done by manual lymphatic drainage of the edematous region followed by the application of compressive bandages with an indication of use throughout the day until evening.

 It is important to know that this method is contraindicated in case of: acute bacterial infections of edematous regions, acute viral infections, deep or superficial thrombosis or in the presence of ulcers after radiotherapy. Light exercise that stimulates lymphatic drainage through muscle contraction is also recommended as a therapeutic method for lymphedema.

In situations where compressive methods cannot be applied (swelling of the neck and head), the lymph taping method is successfully used as a replacement for compressive bandages.

High-performance surgical techniques for treating lymphedema include, among other things, performing anastomoses between the blood and lymph vessels, as well as transplanting the lymph nodes.