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Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series
Objectives—To investigate the clinical characteristics, managements, outcome, and evaluate the risk factors of Multisystem (MS) Langerhans Cell Histiocytosis (LCH) with diverse skin lesions as the first sign in four young infants. Methods—Their clinical features, disease progression, therapy, and ou...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9315804/ https://www.ncbi.nlm.nih.gov/pubmed/35887522 http://dx.doi.org/10.3390/jpm12071024 |
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author | Han, Dan Li, Fei Yahya, Wahid H. Ge, Rui Zhao, Yan Liu, Bei Zhou, Yan Wen, Zhuoyu |
author_facet | Han, Dan Li, Fei Yahya, Wahid H. Ge, Rui Zhao, Yan Liu, Bei Zhou, Yan Wen, Zhuoyu |
author_sort | Han, Dan |
collection | PubMed |
description | Objectives—To investigate the clinical characteristics, managements, outcome, and evaluate the risk factors of Multisystem (MS) Langerhans Cell Histiocytosis (LCH) with diverse skin lesions as the first sign in four young infants. Methods—Their clinical features, disease progression, therapy, and outcomes were reviewed and analyzed retrospectively. Results—The average onset age of skin lesions was about 2 months. Cases 1 and 2 had risk organs involved (RO+) and a lack of bone lesions, and progression could not be reversed by systemic chemotherapy. They both died eventually. Cases 3 and 4 (RO–) had bone involvement and were given systemic chemotherapy for a prolonged duration. Unluckily, Case 3 had a recurrence 2 years later, while Case 4’s recurrence happened nearly one year later, and diabetes insipidus one and a half years later. They both survived and are still in remission. Conclusion—MS-LCH infants with a low age of the first presentation in the skin are prone to dissemination, while RO+ is associated with high mortality. In addition, bone involvement may be a protective factor. Immunohistochemical examination of skin tissue facilitates correct early diagnosis, and adequate follow-up is necessary. |
format | Online Article Text |
id | pubmed-9315804 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-93158042022-07-27 Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series Han, Dan Li, Fei Yahya, Wahid H. Ge, Rui Zhao, Yan Liu, Bei Zhou, Yan Wen, Zhuoyu J Pers Med Article Objectives—To investigate the clinical characteristics, managements, outcome, and evaluate the risk factors of Multisystem (MS) Langerhans Cell Histiocytosis (LCH) with diverse skin lesions as the first sign in four young infants. Methods—Their clinical features, disease progression, therapy, and outcomes were reviewed and analyzed retrospectively. Results—The average onset age of skin lesions was about 2 months. Cases 1 and 2 had risk organs involved (RO+) and a lack of bone lesions, and progression could not be reversed by systemic chemotherapy. They both died eventually. Cases 3 and 4 (RO–) had bone involvement and were given systemic chemotherapy for a prolonged duration. Unluckily, Case 3 had a recurrence 2 years later, while Case 4’s recurrence happened nearly one year later, and diabetes insipidus one and a half years later. They both survived and are still in remission. Conclusion—MS-LCH infants with a low age of the first presentation in the skin are prone to dissemination, while RO+ is associated with high mortality. In addition, bone involvement may be a protective factor. Immunohistochemical examination of skin tissue facilitates correct early diagnosis, and adequate follow-up is necessary. MDPI 2022-06-22 /pmc/articles/PMC9315804/ /pubmed/35887522 http://dx.doi.org/10.3390/jpm12071024 Text en © 2022 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 | Article Han, Dan Li, Fei Yahya, Wahid H. Ge, Rui Zhao, Yan Liu, Bei Zhou, Yan Wen, Zhuoyu Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series |
title | Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series |
title_full | Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series |
title_fullStr | Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series |
title_full_unstemmed | Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series |
title_short | Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series |
title_sort | multisystem langerhans cell histiocytosis in younger infants first presenting in skin: a case series |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9315804/ https://www.ncbi.nlm.nih.gov/pubmed/35887522 http://dx.doi.org/10.3390/jpm12071024 |
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