Differential clinical characteristics of acute liver failure caused by hemophagocytic lymphohistiocytosis in children.

BACKGROUND

Children with acute liver failure (ALF) caused by hemophagocytic lymphohistiocytosis (HLH) may be at risk of undergoing unnecessary liver transplantation (LT). The aim of this study was to compare the characteristics of ALF caused by HLH with those of ALF of unknown etiology in children.

METHODS

The clinical features and laboratory findings for eight children with ALF caused by HLH (ALF-HLH group) and 27 children with ALF of unknown etiology (ALF-UK group) were retrospectively compared by reviewing medical records.

RESULTS

The ALF-HLH group had a higher incidence of pleural effusion, C-reactive protein elevation (especially >5 mg/dL), thrombocytopenia, anemia, fever, splenomegaly, and hypoalbuminemia (<2.5 mg/dL), and a higher in-hospital mortality rate. No significant differences were found in the white blood cell count, liver enzymes, coagulation profile, or incidence of hepatomegaly.

CONCLUSIONS

LT should be performed only after it is proven that ALF is not caused by HLH, if a child with ALF shows the differential clinical features of ALF caused by HLH. Further research with larger sample sizes, however, is needed.