Profound hyperferritinemia may not be specific for the diagnosis of haemophagocytic lymphohistiocytosis (HLH) in Asian children

Abstract

Profound hyperferritinaemia of >10,000 μg/L has been suggested to be highly specific for the diagnosis of paediatric haemophagocytic lymphohistiocytosis (HLH) in western literature but similar studies are lacking in Asian children. We did a single-centre retrospective study analysing paediatric patients with a ferritin value of >500 mcg/L admitted to a children’s hospital in Singapore between January 2010 and December 2015. Sensitivity and specificity of ferritin for diagnosing HLH were analyzed at various ferritin levels. The finding of hyperferritinemia has a much lower specificity for HLH in our Asian paediatric population compared to Western population as it is also a finding in many infective and inflammatory conditions. Thus, while ferritin remains a good screening marker, it is not specific for HLH and other clinical and laboratory parameters should be considered prior to initiating HLH-specific treatment.