We understand that lymphedema occurs when there has been some type of stressor to the lymphatic system in that area. This may be a trauma, removal of portions of the lymphatics, or you maybe could have even been born with an abnormal lymphatic system.
In the case of breast cancer-related lymphedema (BCRL), the problem usually comes from stress to the lymphatic system in the area where the cancer is being treated. When the workload of the lymphatics in that area becomes overwhelming, the system will begin to slow down and work less efficiently, creating swelling.
It helps to understand the anatomy behind the lymphatic system, and how disruption to any of these areas will typically lead lymphedema. Additionally, we'll break down the stages of lymphedema, including signs and symptoms associated with each.
The Anatomy
Briefly, the lymphatic system is made up of multiple vessels, including the following:
- Lymph fluid: Contains mostly proteins, as well as fats, cellular debris, water, white blood cells, and other larger molecules not able to be processed by the venous system until filtered by the lymphatics.
- Lymph nodes: The lymph nodes filter the lymphatic fluid. They allow healthy substances to continue through the lymphatic system, which will eventually enter back into the circulation, and prevent unhealthy substances, like toxins and cellular debris, from moving forward.
- Lymphatic Organs: The lymphatic organs consist of the spleen, thymus, tonsils, bone marrow and adenoids.
- Lymphocytes: Lymphocytes are the primary cells of the lymphatic system. They are white blood cells that help with immune protection.
- Lymphatic Vessels: There is a continuum of lymphatic vessels consisting of lymph capillaries, lymphatic collectors, lymphatic trunks and ducts. These vessels help to transport the lymph fluid throughout the lymphatic system, then eventually back into the venous system.
Stages of Lymphedema
When lymphedema occurs, it is considered a progressive condition. The following are stages used to identify each phase of lymphedema progression.
Stage 0:
This is considered the latent, or dormant, stage of lymphedema. No active swelling is visibly present; however, there is a known risk of potential lymphedema to develop. Symptoms present may include a sensation of heaviness, fatigue or tingling in the at-risk limb. Preventative measures should be taken in this stage.
Stage 1:
In this stage, lymphedema is spontaneously reversible. Swelling is usually intermittent versus constant. There may be pitting present. Swelling will normally resolve with elevation of the affected limb. You should be receiving active treatment in this stage in order to avoid further lymphedema progression.
Stage 2:
In this stage, lymphedema is spontaneously irreversible. Swelling has become more constant and does not resolve as easily with elevation alone. You may begin to notice firmness in the skin, otherwise known as fibrosis. Fibrosis typically occurs as the proteins in the lymphatic fluid begin to accumulate together. More aggressive treatment measures will be needed in this stage. There is a higher risk for development of cellulitis or a blood clot in Stage 2.
Stage 3:
This is the most advanced stage of lymphedema. Commonly, elephantiasis will be present. This involves abnormal folds of skin present along the affected limb. Further skin changes may occur, including development of hyperkeratosis (thickening of the skin), papillomatosis and lymphangiectasia. There is a much higher risk for cellulitis, blood clots, and wound development. Even in this stage, treatment can still be successful, but will need to be aggressive.
References:
- The American Cancer Society medical and editorial content team. What Is Lymphedema? American Cancer Society. Updated May 25, 2021. Accessed September 24, 2022. https://www.cancer.org/treatment/treatments-and-side-effects/physical-side-effects/lymphedema/what-is-lymphedema.html.
- Stages of Lymphedema. National Lymphedema Network. Accessed September 24, 2022. https://lymphnet.org/stages-of-lymphedema.