Hemophagocytic Lymphohistiocytosis in Adults: Overview, Diagnosis, and Treatment / 대한내과학회지
Korean Journal of Medicine
;
: 525-534, 2015.
Article
in Korean
| WPRIM
| ID: wpr-162285
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Autoimmune Diseases
/
Splenomegaly
/
Clinical Protocols
/
Apoptosis
/
Sepsis
/
Hematopoietic Stem Cell Transplantation
/
Diagnosis
/
Drug Therapy
/
Lymphohistiocytosis, Hemophagocytic
/
Macrophage Activation Syndrome
Type of study:
Diagnostic study
/
Practice guideline
Limits:
Adult
/
Humans
Language:
Korean
Journal:
Korean Journal of Medicine
Year:
2015
Type:
Article
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