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 (...

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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
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author Marciniak, Aleksandra
Nawrocka-Rutkowska, Jolanta
Brodowska, Agnieszka
Starczewski, Andrzej
Szydłowska, Iwona
author_facet Marciniak, Aleksandra
Nawrocka-Rutkowska, Jolanta
Brodowska, Agnieszka
Starczewski, Andrzej
Szydłowska, Iwona
author_sort Marciniak, Aleksandra
collection PubMed
description 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.
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spelling pubmed-106720912023-11-12 Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study Marciniak, Aleksandra Nawrocka-Rutkowska, Jolanta Brodowska, Agnieszka Starczewski, Andrzej Szydłowska, Iwona J Pers Med Case Report 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. MDPI 2023-11-12 /pmc/articles/PMC10672091/ /pubmed/38003913 http://dx.doi.org/10.3390/jpm13111598 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Marciniak, Aleksandra
Nawrocka-Rutkowska, Jolanta
Brodowska, Agnieszka
Starczewski, Andrzej
Szydłowska, Iwona
Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
title Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
title_full Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
title_fullStr Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
title_full_unstemmed Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
title_short Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
title_sort lymphangioleiomyomatosis with tuberous sclerosis complex—a case study
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10672091/
https://www.ncbi.nlm.nih.gov/pubmed/38003913
http://dx.doi.org/10.3390/jpm13111598
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