PT - JOURNAL ARTICLE AU - McLean, Josef AU - Katebian, Roia AU - Suh, Eugene AU - Mirza, Kamran AU - Amin, Sachin TI - Neonatal Hemophagocytic Lymphohistiocytosis AID - 10.1542/neo.20-6-e316 DP - 2019 Jun 01 TA - NeoReviews PG - e316--e325 VI - 20 IP - 6 4099 - http://neoreviews.aappublications.org/content/20/6/e316.short 4100 - http://neoreviews.aappublications.org/content/20/6/e316.full SO - NeoReviews2019 Jun 01; 20 AB - Abbreviations:FDA: Food and Drug AdministrationfHLH: familial hemophagocytic lymphohistiocytosisHLH: hemophagocytic lymphohistiocytosisIFNγ: interferon γNK: natural killersCD25/sIL-2R: concentration of soluble interleukin 2 receptorSCT: stem cell transplantationHemophagocytic lymphohistiocytosis (HLH) is extremely rare in the neonatal period. The incidence of neonatal HLH is not confirmed and may range from 1 in 50,000 to 150,000. The incidence varies based on ethnicity, particularly in populations in which consanguinity is common. HLH is associated with a high fatality rate and poor prognosis, making it important to recognize and diagnose it early. This review will concentrate primarily on the diagnosis and management of neonatal HLH.