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

Sporadic lymphangioleiomyomatosis. Presentation of a case of pulmonary disease with retroperitoneal lymphangioleiomyomas

Contenido principal del artículo

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.

 

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Detalles del artículo

Biografía del autor/a (VER)

Claudia Patricia Zuluaga Gómez, MD., Hospital Universitario Mayor Mederi. Universidad del Rosario.

Médica Radióloga. Hospital Universitario Mayor Mederi. Universidad del Rosario.

Astrid Arroyave, MD., Universidad de Antioquia.

Fellow de Imagen Corporal. Universidad de Antioquia.

Diana Carolina Moreno O., MD., Universidad del Rosario

Residente de Medicina Interna. Universidad del Rosario.

Juan Ricardo Lutz, MD., Hospital Universitario Mayor Mederi. Universidad del Rosario.

Médico Neumólogo. Hospital Universitario Mayor Mederi. Universidad del Rosario.

Jorge Alberto Carrillo Bayona, MD., Hospital Universitario Mayor Méderi. Universidad del Rosario.

Médico Radiólogo. Hospital Universitario Mayor Méderi. Universidad del Rosario.

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