Linfangioleiomiomatosis esporádica. Presentación de un caso con enfermedad pulmonar y linfangioleiomiomas retroperitoneales

Claudia Patricia Zuluaga Gómez, MD., Astrid Arroyave, MD., Diana Carolina Moreno O., MD., Juan Ricardo Lutz, MD., Jorge Alberto Carrillo Bayona, MD.

Resumen


La linfangioleiomiomatosis (LAM) es una enfermedad multisistémica asociada a mutaciones del gen TSC 2, que se presenta en forma esporádica (LMA-E) o asociada al complejo de esclerosis tuberosa (LAM-CET). La LAM cursa con enfermedad pulmonar relacionada con quistes en el parénquima. Sin embargo, los pacientes con LAM-E pueden evidenciar alteraciones mediastinales, retroperitoneales y renales, con adenomegalias, linfangioleiomiomas y angiomiolipomas.

Presentamos el caso de una paciente con LAM-E y enfermedad pulmonar quística asociada a linfangioleiomiomas retroperitoneales y pélvicos.

 


Palabras clave


linfangioleiomiomatosis; linfangioleiomiomas; angiomiolipomas; quistes

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Referencias


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DOI: http://dx.doi.org/10.30789/rcneumologia.v27.n2.2015.31

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