Clin Pediatr Hematol Oncol.  2017 Apr;24(1):11-20. 10.15264/cpho.2017.24.1.11.

Hemophagocytic Lymphohistiocytosis

• Park SY
• Lee JM

• KMID: 2379346
• DOI: http://doi.org/10.15264/cpho.2017.24.1.11

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.

Keyword

Hemophagocytic lymphohistiocytosis; Genetics; Pathogenesis; Treatment

MeSH Terms

Diagnosis
Genetics
Humans
Immune System
Immunosuppression
Inflammation
Lymphohistiocytosis, Hemophagocytic*
Treatment Outcome