肺淋巴管肌瘤病lymphangiolei

Lymphangioleiomyomatosis(LAM)isararedisorderresultingfromproliferationinthelung,kidney,andaxiallymphaticsofabnormalsmoothmuscle–likecells(LAMcells)thatexhibitfeaturesofneoplasiaandneuralcrestorigin.Cysticdestructionofthelungwithprogressivepulmonarydysfunctionandthepresenceofabdominaltumors(eg,angiomyolipomas[AML],lymphangioleiomyomas)characterizethedisease.LAMtypicallyoccursinpremenopausalwomen,suggestingtheinvolvementoffemalehormonesindiseasepathogenesis.LAMcanoccurwithincreasedfrequencyinpatientswithtuberoussclerosis







































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