Hemophagocytic lymphohistiocytosis - A fatal cause of persistent fever in neonates: A case series

Primary hemophagocytic lymphohistiocytosis (HLH) is a rare fulminant genetic disease with uncontrolled immune activation and multiorgan involvement. It quite in neonates but high index of suspicion needed as the condition will present like sepsis associated mortality. Persistent fever prominent clinical feature. Genetic diagnosis essential autosomal recessive familial types. Here, we report four cases HLH diagnosed newborn period. We got an uncommon homozygous mutation STXBP2 involving exon 19, which has been reported only very few cases. should be considered differential any sick neonate who presents prolonged unusual course