Hepatic Manifestations of Pediatric Hemophagocytic Lymphohistocytosis

Abstract

emophagocytic lymphohistiocytosis (HLH); is a hyper-inflammatory disorder caused by systemic overgrowth of macrophages in the reticulo-endothelial system leading to cytokine storm; presented mainly with fever, splenomegaly, bi/pancytopenia; hyper-ferritinemia, hyper-triglyceridemia and hypofibrinogenemia; which can progresses early to disseminated intravascular coagulation (DIC), multiorgan dysfunction with dismal outcome. Hepatic manifestations are not well recognized primary presentation in pediatric patients with HLH, thus mandates high levels of suspicious for early diagnosis. Our work aimed at studying the hepatic involvement clinically, laboratory, and pathologically in patients clinically diagnosed or genetically confirmed 1ry HLH and in patients with 2ry acquired HLH. A retrospective cohort study including patients diagnosed clinically as HLH or genetically confirmed 1ry HLH and patients with 2ry HLH was performed. Data was collected from patients files in the Pediatric Hematology oncology Clinic, Children’s Hospital, Ain Shams University during the period from April 2021 to September 2021.