Hemophagocytic lymphohistiocytosis in children

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a heterogenic syndrome characterized by an acute, life-threatening inflammation due to a highly stimulated but ineffective immune response. Depending on the etiology, HLH is divided into primary (genetic) and secondary (acquired) forms. Primary HLH can be divided into familial HLH and HLH associated with other genetic disorders. Secondary HLH usually occurs in the context of a severe infection, rheumatic disease, or malignancy. HLH in children is a rare condition characterized by nonspecific symptoms and poor prognosis. Novel diagnostic tools and therapeutic methods give hope to improve the survival of the patients.