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Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos...

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Detalles Bibliográficos
Autores principales: Kamal, Achmad Fauzi, Luthfi, Andi Praja Wira Yudha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6702438/
https://www.ncbi.nlm.nih.gov/pubmed/31452877
http://dx.doi.org/10.1016/j.amsu.2019.07.030
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author Kamal, Achmad Fauzi
Luthfi, Andi Praja Wira Yudha
author_facet Kamal, Achmad Fauzi
Luthfi, Andi Praja Wira Yudha
author_sort Kamal, Achmad Fauzi
collection PubMed
description BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent. METHODS: We deliver a case of a 10-month-old boy who consulted to our department. Previously misdiagnosed as a Kawasaki syndrome, TORCH, and osteomyelitis. He had undergone several examinations and had been discussed in clinocipathological conference (CPC) to narrow down the diagnosis. RESULT: After serial examinations, the diagnosis of Langerhans Cell Histiocytosis was confirmed and chemotherapy was initiated. And after 6 cycles of chemotherapy, with 1-week interval of each therapy, the clinical appearance of this patient significantly improved. CONCLUSION: Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses for the orthopedic surgeon. By doing a comprehensive examination, it is possible to narrowing down the diagnosis and planning the accurate treatment.
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spelling pubmed-67024382019-08-26 Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report Kamal, Achmad Fauzi Luthfi, Andi Praja Wira Yudha Ann Med Surg (Lond) Case Report BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent. METHODS: We deliver a case of a 10-month-old boy who consulted to our department. Previously misdiagnosed as a Kawasaki syndrome, TORCH, and osteomyelitis. He had undergone several examinations and had been discussed in clinocipathological conference (CPC) to narrow down the diagnosis. RESULT: After serial examinations, the diagnosis of Langerhans Cell Histiocytosis was confirmed and chemotherapy was initiated. And after 6 cycles of chemotherapy, with 1-week interval of each therapy, the clinical appearance of this patient significantly improved. CONCLUSION: Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses for the orthopedic surgeon. By doing a comprehensive examination, it is possible to narrowing down the diagnosis and planning the accurate treatment. Elsevier 2019-08-02 /pmc/articles/PMC6702438/ /pubmed/31452877 http://dx.doi.org/10.1016/j.amsu.2019.07.030 Text en © 2019 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Kamal, Achmad Fauzi
Luthfi, Andi Praja Wira Yudha
Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report
title Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report
title_full Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report
title_fullStr Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report
title_full_unstemmed Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report
title_short Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report
title_sort diagnosis and treatment of langerhans cell histiocytosis with bone lesion in pediatric patient: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6702438/
https://www.ncbi.nlm.nih.gov/pubmed/31452877
http://dx.doi.org/10.1016/j.amsu.2019.07.030
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