Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study

Lymphangioleiomyomatosis (LAM) is characterized by lung cysts that cause lung deterioration, changes in the lymphatic system, and tumors in the kidneys. It mainly affects women of reproductive age and is a progressive disease. LAM can occur as an isolated disease or coexist with tuberous sclerosis (...

Descripción completa

Detalles Bibliográficos
Autores principales: Marciniak, Aleksandra, Nawrocka-Rutkowska, Jolanta, Brodowska, Agnieszka, Starczewski, Andrzej, Szydłowska, Iwona
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10672091/
https://www.ncbi.nlm.nih.gov/pubmed/38003913
http://dx.doi.org/10.3390/jpm13111598
Descripción
Sumario:Lymphangioleiomyomatosis (LAM) is characterized by lung cysts that cause lung deterioration, changes in the lymphatic system, and tumors in the kidneys. It mainly affects women of reproductive age and is a progressive disease. LAM can occur as an isolated disease or coexist with tuberous sclerosis (TSC). The source of LAM cells is unknown. Patients with confirmed LAM should be treated with an mTOR inhibitor, sirolimus, or everolimus. We present a case of LAM with TSC in a patient whose symptoms, including those in the lymph nodes and chyaloperitoneum, mainly concern the abdominal cavity.

Ejemplares similares