Lymphedema pathophysiology

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Lymphatic System Review - The Body's Sewer System

  • Core Function: A one-way drainage network returning interstitial fluid (lymph) and proteins to the venous circulation. Essential for fluid balance, immune cell trafficking, and chylomicron transport from the gut.
  • Driving Force: Primarily driven by intrinsic lymphatic vessel contraction and surrounding skeletal muscle pump action.

High-Yield: Lymphedema is a protein-rich edema. This high protein content incites chronic inflammation, leading to subcutaneous fibrosis and adipose tissue deposition, which is why it often becomes non-pitting over time.

Lymphedema Defined - When the Drains Clog

Lymphedema Pathophysiology: Normal vs. Obstructed Flow

  • Core Defect: Imbalance between lymphatic load & transport capacity, leading to accumulation of protein-rich interstitial fluid.
  • Pathophysiology: Stagnant lymph proteins trigger chronic inflammation, stimulating fibroblasts and adipocytes.
    • Leads to progressive subcutaneous fibrosis and adipose tissue deposition.
    • Results in non-pitting edema over time and ↑ risk of cellulitis.

High-Yield: Chronic lymphedema is characteristically non-pitting due to significant subcutaneous protein deposition and fibrosis. A positive Stemmer sign (inability to pinch the skin at the base of the second toe) is pathognomonic.

Pathophysiology Cascade - The Protein Party Crash

  • Initial Stage: Lymphatic obstruction leads to accumulation of protein-rich interstitial fluid, resulting in soft, pitting edema.
  • Chronic Stage: Stagnant, high-protein fluid incites a chronic inflammatory response.
    • Macrophages release cytokines and growth factors.
    • Fibroblasts and adipocytes are stimulated.
  • Irreversible Changes: Progressive fibrosis (collagen) and adipose tissue deposition occur.
    • Tissue becomes hard, indurated, and non-pitting (brawny edema).
    • ↑ Risk of cellulitis & lymphangitis.

Histology of Lymphedema

Stewart-Treves Syndrome: A rare but classic complication of chronic lymphedema (e.g., post-mastectomy) is lymphangiosarcoma, an aggressive cutaneous angiosarcoma.

Clinical Presentation & Staging - Spotting the Swelling

Lymphedema Staging: Földi, MDACC, ISL

  • Presentation: Insidious onset of painless, progressive swelling.
    • Early stage: Soft, pitting edema that resolves with limb elevation.
    • Late stage: Firm, non-pitting (brawny) edema due to fibrosis and adipose tissue deposition.
  • Key Sign:
    • Positive Stemmer's sign: Inability to pinch or tent the skin on the dorsum of the second toe (or finger).
  • ISL Staging (0-3):
    • Stage 0: Latent/subclinical; impaired transport, no visible swelling.
    • Stage 1: Reversible; pitting edema, resolves with elevation.
    • Stage 2: Spontaneously irreversible; non-pitting edema, fibrosis present.
    • Stage 3: Lymphostatic elephantiasis; significant skin changes (e.g., papillomas, hyperkeratosis).

⭐ Chronic lymphedema is a major risk factor for Stewart-Treves syndrome, a rare but aggressive cutaneous angiosarcoma. Look for a new purple nodule or plaque on the affected limb.

High‑Yield Points - ⚡ Biggest Takeaways

  • Lymphedema is the accumulation of protein-rich interstitial fluid from lymphatic system failure.
  • Primary lymphedema arises from congenital abnormalities like lymphatic aplasia or hypoplasia (e.g., Milroy disease).
  • Secondary lymphedema follows lymphatic obstruction or disruption, commonly from surgery, radiation, or tumors.
  • Worldwide, the most common cause is filariasis (Wuchereria bancrofti).
  • Chronic cases progress to non-pitting edema with subcutaneous fibrosis and hyperkeratosis.
  • Complications include recurrent cellulitis and lymphangiosarcoma (Stewart-Treves syndrome).

Practice Questions: Lymphedema pathophysiology

Test your understanding with these related questions

A 52-year-old man presents to the emergency department because of pain and swelling in his left leg over the past few hours. He traveled from Sydney to Los Angeles 2 days ago. He has had type 2 diabetes mellitus for 10 years and takes metformin for it. He has smoked a pack of cigarettes daily for 25 years. His temperature is 36.9°C (98.4°F), the blood pressure is 140/90 mm Hg, and the pulse is 90/min. On examination, the left calf is 5 cm greater in circumference than the right. The left leg appears more erythematous than the right with dilated superficial veins. Venous duplex ultrasound shows non-compressibility. Which of the following best represents the mechanism of this patient’s illness?

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Flashcards: Lymphedema pathophysiology

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What dermatome is found at the xiphoid process? _____

TAP TO REVEAL ANSWER

What dermatome is found at the xiphoid process? _____

T7

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