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Review Article
A Recent Update on Histiocytic Disorder in Children: Focus on Diagnosis and Treatment
Clin Pediatr Hematol Oncol 2020;27:32-42.
Published online April 30, 2020
© 2020 Korean Society of Pediatric Hematology-Oncology

Hoi Soo Yoon

Department of Pediatrics, Kyung Hee University College of Medicine, Seoul, Korea
Correspondence to: Hoi Soo Yoon
Department of Pediatrics, Kyung Hee University College of Medicine, 23 Kyungheedae-ro, Dongdaemungu, Seoul 02447, Korea
Tel: +82-2-958-8206
Fax: +82-2-958-8304
E-mail: snoopyi@hanmail.net
ORCID ID: orcid.org/0000-0003-1688-3226
Received April 6, 2020; Revised April 13, 2020; Accepted April 21, 2020.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
The histiocytosis is rare disorder characterized by the accumulation of macrophages, dendritic cells, or monocyte-derived cells in various tissues and organs of children and adults. Classifying histiocytic disorders is difficult and has changed over time as an understanding of the biology of these cells has evolved. The most recently revised 2016 WHO classification of histiocytosis and neoplasms of the macrophage-dendritic cell lineages has proposed grouping this diverse group of over 100 clinical entities into five main groups based on clinical, histologic, and molecular relevance. Comprehensive genomic studies for histiocytosis have been described and our understanding of the pathogenesis and biology has increased over the past decade. These advances will be able to make precision medicine and targeted therapy possible in patients with histiocytosis. Among the histiocytosis, this review mainly focuses on the updated diagnosis and treatment of Langerhans cell histiocytosis (LCH) and hemophagocytic lymphohistiocytosis (HLH) in children.
Keywords: Histiocytic disorder, Langerhans cell histiocytosis, Hemophagocytic lymphohistiocytosis, Children
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April 2020, 27 (1)
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  • Hoi Soo Yoon