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Sporadic Lymphangioleiomyomatosis Disease: A Case Report

Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease of the lung and lymphatic system that primarily affects women of childbearing age. LAM is a progressive disease with a terrible prognosis, which worsens over time and is extremely difficult to treat. In this study, we discuss the case of a 3...

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Autores principales: Nikmanesh, Yousef, Shokripour, Mansoureh, Mokhtari, Maral, Khazayi, Mahdi, Monabati, Ahmad, Rezayi, Ramin, Bahtouee, Mehrzad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Shiraz University of Medical Sciences 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10541542/
https://www.ncbi.nlm.nih.gov/pubmed/37786468
http://dx.doi.org/10.30476/ijms.2022.95521.2689
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author Nikmanesh, Yousef
Shokripour, Mansoureh
Mokhtari, Maral
Khazayi, Mahdi
Monabati, Ahmad
Rezayi, Ramin
Bahtouee, Mehrzad
author_facet Nikmanesh, Yousef
Shokripour, Mansoureh
Mokhtari, Maral
Khazayi, Mahdi
Monabati, Ahmad
Rezayi, Ramin
Bahtouee, Mehrzad
author_sort Nikmanesh, Yousef
collection PubMed
description Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease of the lung and lymphatic system that primarily affects women of childbearing age. LAM is a progressive disease with a terrible prognosis, which worsens over time and is extremely difficult to treat. In this study, we discuss the case of a 31-year-old woman with LAM who was initially misdiagnosed with leiomyoma and the way that led to a true diagnosis and effective treatment. Following a precise diagnosis based on comprehensive clinical data and particular immunohistochemical tests, sirolimus treatment was initiated, and the patient entirely responded to the treatment. This case report demonstrated that LAM is an uncommon condition that is challenging to diagnose, which causes its treatment to be delayed.
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spelling pubmed-105415422023-10-02 Sporadic Lymphangioleiomyomatosis Disease: A Case Report Nikmanesh, Yousef Shokripour, Mansoureh Mokhtari, Maral Khazayi, Mahdi Monabati, Ahmad Rezayi, Ramin Bahtouee, Mehrzad Iran J Med Sci Case Report Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease of the lung and lymphatic system that primarily affects women of childbearing age. LAM is a progressive disease with a terrible prognosis, which worsens over time and is extremely difficult to treat. In this study, we discuss the case of a 31-year-old woman with LAM who was initially misdiagnosed with leiomyoma and the way that led to a true diagnosis and effective treatment. Following a precise diagnosis based on comprehensive clinical data and particular immunohistochemical tests, sirolimus treatment was initiated, and the patient entirely responded to the treatment. This case report demonstrated that LAM is an uncommon condition that is challenging to diagnose, which causes its treatment to be delayed. Shiraz University of Medical Sciences 2023-09 /pmc/articles/PMC10541542/ /pubmed/37786468 http://dx.doi.org/10.30476/ijms.2022.95521.2689 Text en Copyright: © Iranian Journal of Medical Sciences https://creativecommons.org/licenses/by-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-NoDerivatives 4.0 International License. This license allows reusers to copy and distribute the material in any medium or format in unadapted form only, and only so long as attribution is given to the creator. The license allows for commercial use.
spellingShingle Case Report
Nikmanesh, Yousef
Shokripour, Mansoureh
Mokhtari, Maral
Khazayi, Mahdi
Monabati, Ahmad
Rezayi, Ramin
Bahtouee, Mehrzad
Sporadic Lymphangioleiomyomatosis Disease: A Case Report
title Sporadic Lymphangioleiomyomatosis Disease: A Case Report
title_full Sporadic Lymphangioleiomyomatosis Disease: A Case Report
title_fullStr Sporadic Lymphangioleiomyomatosis Disease: A Case Report
title_full_unstemmed Sporadic Lymphangioleiomyomatosis Disease: A Case Report
title_short Sporadic Lymphangioleiomyomatosis Disease: A Case Report
title_sort sporadic lymphangioleiomyomatosis disease: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10541542/
https://www.ncbi.nlm.nih.gov/pubmed/37786468
http://dx.doi.org/10.30476/ijms.2022.95521.2689
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