Hemophagocytic Lymphystiocytosis (HLH) in pediatrics; new therapeutic options.

Abstract

Abstract

Bacground: Hemophagocytic lymphohistiocytosis (HLH) is a pathology with a high mortality rate, characterized by a variable clinical presentation with rapid progression to multiorgan failure without a clear incidence reported in Latin America, probably due to the lack of diagnostic suspicion, its triggers cause an exaggerated immune response with cytokine storm responsible for the clinical picture.  Treatment is currently managed according to the 1994-2004 HLH protocol, however, new research in the last decade has been conducted with less aggressive therapeutic agents.

Objective: Update the literature and new therapeutic options on hemophagocytic lymphohistiocytosis. 

Methodology: A bibliographic search was performed in databases like: PUBMED, MEDLINE, COCHRANE, and articles were obtained from medical journals such as: THE JOURNAL OF IMMUNOLOGY, BMC PEDIATRICS, PEDIATRIC RHEUMATOLOGY, JOURNAL OF MEDICAL, BMC INFECTIOUS DISEASES.

Results: The therapy established by the Histiocyte Society in the HLH-2004 study remains the standard therapy for hemophagocytic lymphohistiocytosis, if co-infection by Epstein Barr virus is confirmed, the monoclonal antibody CD20-Rituximab should be initiated, immunomodulatory therapy showed favorable results, however studies in larger populations are lacking to establish its use as initial therapy, however it could be used as adjuvant therapy.

Conclusions: Hemophagocytic lymphohistiocytosis is a pathology with a high mortality rate, diagnostic suspicion and early initiation of treatment improves the survival rate.