Korean J Med.  2015 May;88(5):525-534. 10.3904/kjm.2015.88.5.525.

Hemophagocytic Lymphohistiocytosis in Adults: Overview, Diagnosis, and Treatment

• Kim DY

• KMID: 1834039
• DOI: http://doi.org/10.3904/kjm.2015.88.5.525

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.

Keyword

Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome

MeSH Terms

Adult*
Apoptosis
Autoimmune Diseases
Clinical Protocols
Diagnosis*
Drug Therapy
Fever
Hematopoietic Stem Cell Transplantation
Humans
Lymphohistiocytosis, Hemophagocytic*
Macrophage Activation Syndrome
Sepsis
Splenomegaly