Successful early plasma exchange therapy in hemophagocytic lymphohistiocytosis due to hyperacute liver failure; A case report and review of published cases.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare severe inflammatory syndrome of excessive cytokine production. A 16-year-old girl presented with hyperacute liver failure due to idiopathic HLH. Liver failure due to HLH is uncommon, and survival in an adult after hyperacute liver failure is rare. Early diagnosis of the disease and timely treatment with plasma exchange followed by immunosuppressive therapy were associated with the survival of this patient.