Characteristics and management of patients with hemophagocytic lymphohistiocytosis masquerading as liver failure

Liver failure (LF) is a lethal condition characterized by jaundice, coagulation disorders. patients with LF carry a high mortality. Hemophagocytic lymphohistiocytosis (HLH) is established as a rare cause of LF. However, the clinical course of HLH may not be familiar to most hepatologist. We aimed to decipher the characteristics of these patients, to provide experience in the diagnose and management of patients with HLH masquerading as LF. Nine patients initially diagnosed with LF and finally developed HLH between January 2017 and October 2020 were retrospectively included. 78% cases developed LF with no discernable causes. Unexplained cytopenia and recurrent fever with LF may be a red flag for suspicion of HLH. Besides, female maybe predispositional to HLH in the context of LF. Modified DEP regimen was effective to control the fatal inflammatory state, all 9 cases achieved response after only one course. Further, three courses of treatment could substantially make the blood count and liver function return to normalcy, and without recurrence of HLH.