ABSTRACT

Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic condition characterized by ac-tivation of lymphohistiocytosis, leading to cytokine overproduction. The main clinical futures of HLH are prolonged fever and hepatosplenomegaly. Laboratory findings include pancytopenia, elevated triglycerides, ferritin and low fibrinogen. Hepatic dysfunction is often present but initial presentation as hepatic failure is a rare condition. In this report, we described a selected group of patients with dramatic onset, whose first symptom was hepatic failure. After a while, other symp-toms of HLH developed and all children were diagnosed with secondary HLH based on clinical and laboratory criteria. The patients received HLH 94 chemotherapy protocol. They did not go into remission and all patients died due to progressive multiorgan failure. HLH is frequently, rapidly fatal disorder and the main prognostic factor is accurate, early recognition and therapy. For this reason, HLH should be considered in the differential diagnosis of hepatic failure especially if it is concurrent with cytopenias and unexplained prolonged fever.