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Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study

Background and Objectives: Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells. LCH often involves the bone, and its clinical evidence is limited. The purpose of this study is to report on the treatment...

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Autores principales: Hashimoto, Kazuhiko, Nishimura, Shunji, Sakata, Naoki, Inoue, Masami, Sawada, Akihisa, Akagi, Masao
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8067819/
https://www.ncbi.nlm.nih.gov/pubmed/33917120
http://dx.doi.org/10.3390/medicina57040356
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author Hashimoto, Kazuhiko
Nishimura, Shunji
Sakata, Naoki
Inoue, Masami
Sawada, Akihisa
Akagi, Masao
author_facet Hashimoto, Kazuhiko
Nishimura, Shunji
Sakata, Naoki
Inoue, Masami
Sawada, Akihisa
Akagi, Masao
author_sort Hashimoto, Kazuhiko
collection PubMed
description Background and Objectives: Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells. LCH often involves the bone, and its clinical evidence is limited. The purpose of this study is to report on the treatment of LCH at our institution and to add to the evidence for LCH. Materials and Methods: We reviewed six cases of LCH treated in our hospital between November 2005 and February 2016. Patient age at the first visit, sex, site of origin, symptoms, image tools used for diagnosis, biopsy site, complications, treatment, and final clinical outcome were evaluated. The median follow-up period was 41 months. Results: The median patient age at the first visit was 13.5 years. Three male and three female individuals were enrolled. Multiple lesions were observed in five cases, and a solitary lesion was observed in one case. Pain was the chief complaint in five cases. Radiography was the most commonly used imaging tool. Bone scintigraphy or magnetic resonance imaging and positron emission tomography-computed tomography were also used to diagnose systematic LCH. Biopsy of the femur was performed in two cases, and biopsy of the tibia, lumbar vertebrae, rib, and radius was performed in one case each. Regarding comorbidities, one case of hepatitis B and one case of autism were observed. Chemotherapy was initiated in two patients. The other four patients were observed naturally. Continuous disease-free survival was observed in five patients. One patient remained alive but not without disease during the final follow-up examination. Conclusion: LCH should be diagnosed as early as possible to treat it appropriately.
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spelling pubmed-80678192021-04-25 Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study Hashimoto, Kazuhiko Nishimura, Shunji Sakata, Naoki Inoue, Masami Sawada, Akihisa Akagi, Masao Medicina (Kaunas) Article Background and Objectives: Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells. LCH often involves the bone, and its clinical evidence is limited. The purpose of this study is to report on the treatment of LCH at our institution and to add to the evidence for LCH. Materials and Methods: We reviewed six cases of LCH treated in our hospital between November 2005 and February 2016. Patient age at the first visit, sex, site of origin, symptoms, image tools used for diagnosis, biopsy site, complications, treatment, and final clinical outcome were evaluated. The median follow-up period was 41 months. Results: The median patient age at the first visit was 13.5 years. Three male and three female individuals were enrolled. Multiple lesions were observed in five cases, and a solitary lesion was observed in one case. Pain was the chief complaint in five cases. Radiography was the most commonly used imaging tool. Bone scintigraphy or magnetic resonance imaging and positron emission tomography-computed tomography were also used to diagnose systematic LCH. Biopsy of the femur was performed in two cases, and biopsy of the tibia, lumbar vertebrae, rib, and radius was performed in one case each. Regarding comorbidities, one case of hepatitis B and one case of autism were observed. Chemotherapy was initiated in two patients. The other four patients were observed naturally. Continuous disease-free survival was observed in five patients. One patient remained alive but not without disease during the final follow-up examination. Conclusion: LCH should be diagnosed as early as possible to treat it appropriately. MDPI 2021-04-07 /pmc/articles/PMC8067819/ /pubmed/33917120 http://dx.doi.org/10.3390/medicina57040356 Text en © 2021 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
Hashimoto, Kazuhiko
Nishimura, Shunji
Sakata, Naoki
Inoue, Masami
Sawada, Akihisa
Akagi, Masao
Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study
title Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study
title_full Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study
title_fullStr Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study
title_full_unstemmed Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study
title_short Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study
title_sort treatment outcomes of langerhans cell histiocytosis: a retrospective study
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8067819/
https://www.ncbi.nlm.nih.gov/pubmed/33917120
http://dx.doi.org/10.3390/medicina57040356
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