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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...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Shiraz University of Medical Sciences
2023
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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. |
format | Online Article Text |
id | pubmed-10541542 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Shiraz University of Medical Sciences |
record_format | MEDLINE/PubMed |
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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