Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in <scp>K</scp>orea: report of a nationwide survey from <scp>K</scp>orea <scp>H</scp>istiocytosis <scp>W</scp>orking <scp>P</scp>arty

Abstract

Our study showed the unique predominance of a UNC13D mutation and vulnerability to Epstein-Barr virus infection in Korean children with HLH and emphasizes the prognostic significance of age, liver dysfunction, and treatment responses in this disease. A multicenter prospective trial that builds on the present results is warranted to identify subgroups of patients with a poor prognosis and identify optimal treatments.