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Korean J Med > Volume 88(5); 2015 > Article
혈액
The Korean Journal of Medicine 2015;88(5): 525-534.
성인 혈구탐식성림프조직구증식증의 이해, 진단 및 치료
김대영
울산대학교 의과대학 서울아산병원 혈액내과
Hemophagocytic Lymphohistiocytosis in Adults – Overview, Diagnosis, and Treatment
Dae-Young Kim
Department of Hematology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Dae-Young Kim ,Tel: +82-2-3010-5930, Fax: +82-2-3010-6885, Email: dani@amc.seoul.kr
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome involving defective apoptosis in which the pathways regulating the termination of immune and inflammatory responses are disrupted. Fever, cytopenia, splenomegaly, and hemophagocytosis are typical findings of this syndrome. HLH can be induced by genetic disorders (familial) or secondary causes. While familial HLH is rare, secondary causes include infection, autoimmune disease, and malignancy in adults. Adult onset HLH may be confused with or misdiagnosed as sepsis or macrophage activation syndrome due to similar clinical manifestations and laboratory findings. Consequently, it is difficult to diagnose HLH promptly to initiate adequate immunosuppressive treatment or chemotherapy. A pediatric HLH treatment protocol such as HLH-2004 or multi-agent chemotherapy can be given to adults after adjusting the drug dosage and type. After the initial treatment, refractory or reactivated patients should undergo allogenic hematopoietic stem cell transplantation as soon as possible to improve survival. Clinical trials should determine more suitable therapeutic options for adults with HLH.
Keywords: Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome
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