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What are the two main causes of lymphoedema?

4 min read

An estimated 140 to 250 million people worldwide are affected by lymphedema, a chronic swelling condition. A key step toward understanding this condition is knowing what are the two main causes of lymphoedema, which are categorized as either primary or secondary in nature.

Quick Summary

Lymphoedema is caused by two main factors: a congenital or genetic abnormality affecting the lymphatic system's development (primary lymphoedema) or acquired damage to a healthy lymphatic system from external factors like surgery, infection, or trauma (secondary lymphoedema).

Key Points

  • Genetic vs. Acquired: The two main causes of lymphoedema are genetic abnormalities (primary) or damage to a healthy lymphatic system (secondary).

  • Primary is Rare: Primary lymphoedema is uncommon and results from a congenital malformation of the lymphatic system, though it may not appear until later in life.

  • Secondary is Common: Secondary lymphoedema is the more prevalent type, often caused by cancer treatments involving lymph node removal or radiation.

  • Multiple Triggers: Other causes of secondary lymphoedema include severe infections, physical trauma, and chronic conditions like venous disease and obesity.

  • Key Distinction for Management: Knowing whether the cause is primary or secondary is crucial for accurate diagnosis and for developing the most effective long-term management plan.

  • Management, Not Cure: While there is no cure, symptoms can be effectively managed through specialized therapies like compression, manual lymph drainage, and exercise.

In This Article

Understanding the Lymphatic System

Before delving into its causes, it's essential to understand the lymphatic system's role. It is a vital part of your immune system and circulatory system, consisting of a network of vessels, nodes, and organs. Its primary function is to collect and filter excess fluid, called lymph, from your body's tissues and return it to the bloodstream. When this process is disrupted due to a blockage or damage, lymph fluid can accumulate, leading to the swelling known as lymphoedema.

The Two Main Types of Lymphoedema

Experts in lymphoedema categorize the condition based on its origin, which gives rise to the two primary causes. These are primary (genetic) and secondary (acquired) lymphoedema. Distinguishing between these two types is critical for accurate diagnosis and effective management, though the symptoms can often appear similar.

Primary Lymphoedema: A Genetic Origin

Primary lymphoedema is the less common of the two types and results from a genetic mutation that affects the development or function of the lymphatic system. This abnormality can be present from birth but may not manifest as swelling until later in life.

  • Congenital Lymphoedema (Milroy's disease): Present at birth or within the first two years of life, this is often linked to a specific gene mutation.
  • Lymphedema Praecox (Meige disease): The most common form of primary lymphoedema, appearing during puberty, most often in girls, and usually affecting the legs.
  • Lymphedema Tarda: This form begins later in life, typically after age 35.

Secondary Lymphoedema: Acquired Damage

Secondary lymphoedema is the more common type and occurs when a previously healthy lymphatic system is damaged by external factors. The damage can happen suddenly or over time, and its effects can appear years after the initial incident.

Key triggers for secondary lymphoedema include:

  • Cancer Treatment: One of the most prevalent causes in developed countries, particularly after breast cancer treatment. Surgery involving lymph node removal (lymphadenectomy) and radiation therapy can both damage the lymphatic vessels and nodes.
  • Infection: Infections, particularly severe ones, can damage lymphatic vessels and cause scarring. The most common cause worldwide is lymphatic filariasis, a parasitic infection caused by mosquito-transmitted worms that clog the lymph nodes.
  • Trauma or Injury: Accidental injury, burns, or extensive bruising can disrupt the flow of lymph, leading to localized swelling.
  • Obesity: Excess weight, especially severe obesity, increases pressure on lymphatic vessels and is a known risk factor.
  • Venous Disease: Conditions affecting blood flow in the veins, such as deep vein thrombosis (DVT) or chronic venous insufficiency, can overwhelm the lymphatic system, leading to lymphoedema.

Comparing Primary vs. Secondary Lymphoedema

Feature Primary Lymphoedema Secondary Lymphoedema
Cause Genetic mutations causing malformation of the lymphatic system. Damage to a healthy lymphatic system.
Onset At birth, puberty, or later in life. Can occur anytime after an external injury, infection, or treatment.
Prevalence Less common, accounts for a small percentage of cases. Much more common, especially in areas with specific infectious diseases or high rates of cancer treatment.
Hereditary Link Often runs in families, though not always. Not hereditary, though some may have a predisposition.
Common Examples Milroy's disease, Meige disease. Post-cancer surgery lymphedema, filariasis.

Recognizing Symptoms and Risk Factors

Identifying the early signs of lymphoedema is important for management. The most common symptom is swelling, but other indicators may be present.

Symptoms to watch for include:

  • A feeling of heaviness or fullness in an arm or leg.
  • Persistent swelling that may or may not leave a dent when pressed.
  • Tightness of the skin, clothing, or jewelry.
  • Aching, tingling, or discomfort in the affected limb.
  • Thickening and hardening of the skin over time.
  • Repeated infections (cellulitis) in the affected limb.

Understanding your risk factors is a key part of prevention and early detection. Beyond cancer treatment, which is a major factor, older age, obesity, and certain forms of arthritis can also increase the risk of developing lymphedema.

Management and Outlook

While there is currently no cure for lymphoedema, the condition can be effectively managed to reduce symptoms and improve quality of life. Treatment is typically centered around Complete Decongestive Therapy (CDT), which consists of several components:

  1. Manual Lymph Drainage: A specialized massage technique to redirect lymph fluid toward functioning lymph nodes.
  2. Compression Therapy: Using bandages or custom-fitted garments to apply pressure and prevent fluid buildup.
  3. Therapeutic Exercise: Gentle exercises that promote lymphatic circulation through muscle contractions.
  4. Skin Care: Diligent skin care to prevent dryness, cracks, and infections, which can worsen the condition.

In some cases, surgical options, such as lymph node transplant or lymphovenous bypass, may be explored for advanced or persistent cases.

For more detailed information on living with and managing lymphedema, consult the National Cancer Institute's guide on the topic: Lymphedema and Cancer.

Conclusion

The two primary causes of lymphoedema are its classification as either a congenital condition (primary) or one resulting from damage to the lymphatic system (secondary). While the underlying reason dictates the type, proper management and early intervention are paramount for controlling symptoms, reducing infection risk, and enhancing overall well-being. Understanding the specific cause is the first step toward a personalized and effective long-term treatment plan.

Frequently Asked Questions

The most common cause of lymphedema worldwide is lymphatic filariasis, a parasitic infection transmitted by mosquitoes, especially in tropical regions.

Yes, secondary lymphedema can develop soon after treatment or months, or even years, after the cancer treatment has been completed.

While often genetic, primary lymphedema is caused by genetic mutations, but it doesn't always have a clear family history and can sometimes arise spontaneously.

Yes, severe obesity can cause or contribute to secondary lymphedema, as extra fatty tissue can put pressure on and affect the function of lymphatic channels.

A doctor will perform a physical exam, take a medical history, and may use imaging tests like MRI or lymphoscintigraphy to determine if the cause is primary or secondary.

Infections like cellulitis can be both. An untreated, severe infection can damage the lymphatic system, causing lymphedema. Additionally, lymphedema makes a person more susceptible to recurrent skin infections.

Lymphedema is a chronic condition, but with proper management, including therapies like compression and manual lymph drainage, the swelling can be significantly controlled and improved.

References

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Medical Disclaimer

This content is for informational purposes only and should not replace professional medical advice.