CTICasesAlpha-1 Antitrypsin Deficiency Emphysema
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Imaging findings
CT demonstrates confluent emphysematous holes, particularly in the left lower lobe, with very spindly vessels and large voids without intervening normal lung tissue. The characteristic centrilobular emphysema is also noted, but the panlobular destruction at the bases is most prominent.
Key takeaways
This case serves to contrast with LAM, where vessels in cystic areas may appear larger due to compressed normal tissue. In alpha-1 antitrypsin deficiency, the profound panlobular destruction leads to a more complete loss of capillary bed and spindly vessels, distinguishing it from LAM. The absence of normal intervening tissue and large voids are characteristic.
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