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Review Article
Hemophagocytic Lymphohistiocytosis
Clin Pediatr Hematol Oncol 2017;24:11-20.
Published online April 30, 2017
© 2017 Korean Society of Pediatric Hematology-Oncology

Sun Young Park, M.D. and Jae Min Lee, M.D.

Department of Pediatrics, College of Medicine, Yeungnam University, Daegu, Korea
Correspondence to: Jae Min Lee Department of Pediatrics, Yeungnam University Hospital, 170, Hyeonchung-ro, Nam-gu, Daegu 42415, Korea Tel: +82-53-620-3530 Fax: +82-53-629-2252 E-mail: mopic@yu.ac.kr ORCID ID: orcid.org/0000-0001-6822-1051
Received March 26, 2017; Revised April 7, 2017; Accepted April 21, 2017.
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
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disease caused by dysregulated immune responses and overwhelming inflammation to infectious or other triggers of the immune system. HLH may be inherited (primary) or may be secondary to any severe infection, malignancy or rheumatologic disease. Clinical progress of HLH is usually rapid and fatal. Early recognition and assessment of potential causes of HLH is critical to improve survival. Urgent treatment is needed for immunosuppression and degradation of the activated antigen. Over the last two decades, research on genetics and pathophysiology of HLH has much improved treatment outcome of the patient. Here, we review recent advances in our understanding of etiology, pathophysiology, diagnosis and treatment of HLH.
Keywords: Hemophagocytic lymphohistiocytosis, Genetics, Pathogenesis, Treatment
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  • Jae Min Lee