FlashPath - Lung - Pulmonary Lymphangiectasis
Download as PPTX, PDF0 likes148 views
Pulmonary lymphangiectasia is a rare disease characterized by abnormal dilatation of pulmonary lymphatics without an increase in their number. It presents early in life with respiratory distress, hypoxia, and cyanosis. Bilateral chylothorax is common. Microscopy shows multiple cystic spaces lined by flat endothelial cells that expand the surrounding connective tissue in sub-pleural, interlobular septa, and peri-bronchovascular areas. Immunohistochemistry staining is positive for vascular and lymphatic markers like CD31, CD34, D2-40, which helps differentiate it from other cystic lung diseases.
FlashPath - Lung - Pulmonary Lymphangiectasis
- 1. FLASHPATH H a z e m A l i
- 2. PULMONARY LYMPHANGIECTASIS H a z e m A l i
- 3. CLINICAL • Rare disease characterized by abnormal dilatation of pulmonary lymphatics, but without proliferation (no increase in lymphatics number) • Present early in life with neonatal respiratory distress, hypoxia and cyanosis • Bilateral Chylothorax is common finding • Often fatal (poor prognosis)
- 4. CLINICAL Either primary or secondary (Faul Classification): • Primary: – Primary developmental defect of the pulmonary lymphatics • Secondary: – Secondary disorders that impair lymphatic drainage / increase lymph production • including surgery, radiation, infection, tumor, trauma
- 5. GROSS • Firm, heavy and noncompliant lungs • Bilateral milky (lymph) pleural effusion can be seen • Numerous dilated cystic spaces (lymphatics) seen in: – Sub-pleura – Interlobular septa – Peri-bronchovascular
- 6. MICROSCOPY • Pattern: – Multiple cystic spaces (dilated lymphatic vessels) – Lined by flat endothelial cells – Expand (widen) surrounding connective tissue • Surrounding tissues: – Sub-pleural – Interlobular septa – Peri-bronchovascular
- 7. SPECIAL STUDIES • Endothelial cells are positive to Vascular markers – Commonly used: CD31, CD34, Factor VIII • “Cytoplasmic and Membranous” • CD31 is the most sensitive and specific vascular marker – Less commonly used: Ulex europaeus I, CD141 (thrombomodulin), Fli-1 • Also positive to D2-40 (Podoplanin) – “Membranous” – Specific to lymphatic endothelium
- 8. DIFFERENTIAL DIAGNOSIS • Pulmonary Lymphangiomatosis: – Expansion of sub-pleural, Interlobular septa, and peri-bronchovascular connective tissues by: • Increased number of dilated, anastomosing lymphatic channels • Pulmonary interstitial emphysema: – Expansion of sub-pleural, Interlobular septa, and peri-bronchovascular connective tissues by multiple cystic spaces that: • Contain air (not lymph) • Do NOT have lining (negative for vascular markers and D2-40)
- 9. DIFFERENTIAL DIAGNOSIS “ O t h e r c y s t i c l u n g d i s e a s e s ” • Congenital: – Pulmonary sequestration – Congenital pulmonary cysts – Congenital pulmonary airway malformation – Congenital lobar emphysema • Acquired: – Emphysema – Healed abscess – Honeycombing • Mixed: – Cystic fibrosis
- 10. WWW. DO NOT FORGET TO SEARCH FOR MORE PICS AND VIRTUAL SLIDES
- 11. THANK YOU H a z e m A l i