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Review
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Korean J Med. 2015;88(5):525-534. Published online May 1, 2015.
DOI: https://doi.org/10.3904/kjm.2015.88.5.525
- 성인 혈구탐식성림프조직구증식증의 이해, 진단 및 치료
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김대영
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울산대학교 의과대학 서울아산병원 혈액내과
- Hemophagocytic Lymphohistiocytosis in Adults – Overview, Diagnosis, and Treatment
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Dae-Young Kim
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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
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߽ɾ :혈구탐식성림프조직구증식증; 혈구탐식증
- 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
