Advances in the management of hemophagocytic lymphohistiocytosis.

摘要: Hemophagocytic lymphohistiocytosis (HLH) is a prototype of the hemophagocytic syndrome and occurs most often in children. Progress cytokine research has now made it possible to show that HLH as consequence uncontrolled, dysregulated cellular immune reactivity caused by number different underlying diseases. Three major risk groups can be identified: (1) familial (FHL), (2) Epstein-Barr virus-associated (EBV-HLH), (3) life-threatening infection-associated or disease-unknown infancy. Diagnostic criteria exist allow differential diagnosis these groups, which important because distinct therapeutic measures are advised for each group. FHL patients require immediate application immunochemotherapy with core combination corticosteroids etoposide together monitoring central nervous system disease early repeated magnetic resonance imaging brain, followed timely stem cell transplantation (SCT). EBV-HLH should also treated etoposide. Aggressive relapsed cases cyclosporin A and, if necessary, more intensive chemotherapy, such used non-Hodgkin's lymphoma. SCT may needed refractory cases. In herpes simplex virus, adenovirus 7, other pathogen-undetermined infancy, great importance administer appropriate antiviral antibacterial agents. The point make regarding treatment cause must promptly established enable rapid therapy. Currently, 30% 40% have poor outcome. It necessary hematologists cooperate specialists fields so diagnosis, critical improvements outcome, made.