Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study

<i>Background and Objectives</i>: 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 repo...

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Main Authors: Kazuhiko Hashimoto, Shunji Nishimura, Naoki Sakata, Masami Inoue, Akihisa Sawada, Masao Akagi
Format: Article
Language:English
Published: MDPI AG 2021-04-01
Series:Medicina
Subjects:
Online Access:https://www.mdpi.com/1648-9144/57/4/356
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author Kazuhiko Hashimoto
Shunji Nishimura
Naoki Sakata
Masami Inoue
Akihisa Sawada
Masao Akagi
author_facet Kazuhiko Hashimoto
Shunji Nishimura
Naoki Sakata
Masami Inoue
Akihisa Sawada
Masao Akagi
author_sort Kazuhiko Hashimoto
collection DOAJ
description <i>Background and Objectives</i>: 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. <i>Materials and Methods</i>: 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. <i>Results</i>: 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. <i>Conclusion</i>: LCH should be diagnosed as early as possible to treat it appropriately.
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spelling doaj.art-05208e4f2080471bbc11548c189b37712023-11-21T14:32:46ZengMDPI AGMedicina1010-660X1648-91442021-04-0157435610.3390/medicina57040356Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective StudyKazuhiko Hashimoto0Shunji Nishimura1Naoki Sakata2Masami Inoue3Akihisa Sawada4Masao Akagi5Department of Orthopedic Surgery, Kindai University Hospital, Osaka-Sayama City, Osaka 589-8511, JapanDepartment of Orthopedic Surgery, Kindai University Hospital, Osaka-Sayama City, Osaka 589-8511, JapanDepartment of Pediatrics, Kindai University Hospital, Osaka-Sayama City, Osaka 589-8511, JapanDepartment of Hematology/Oncology, Osaka Women’s and Children’s Hospital, Izumi City, Osaka 594-1101, JapanDepartment of Hematology/Oncology, Osaka Women’s and Children’s Hospital, Izumi City, Osaka 594-1101, JapanDepartment of Orthopedic Surgery, Kindai University Hospital, Osaka-Sayama City, Osaka 589-8511, Japan<i>Background and Objectives</i>: 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. <i>Materials and Methods</i>: 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. <i>Results</i>: 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. <i>Conclusion</i>: LCH should be diagnosed as early as possible to treat it appropriately.https://www.mdpi.com/1648-9144/57/4/356Langerhans cellshistiocytosisoutcomes
spellingShingle Kazuhiko Hashimoto
Shunji Nishimura
Naoki Sakata
Masami Inoue
Akihisa Sawada
Masao Akagi
Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study
Medicina
Langerhans cells
histiocytosis
outcomes
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 Langerhans cells
histiocytosis
outcomes
url https://www.mdpi.com/1648-9144/57/4/356
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