In this study, we compared the performances of adapted hemophagocytic lymphohistiocytosis (HLH)-2004 guidelines with those of the new diagnostic H-score to identify patients with HLH in a multicenter cohort consisting of adult and pediatric cases of suspected HLH.
The study sample consisted of 147 cases, including 20 adults and 16 children with HLH. Two sets of biological data were evaluated: at presentation and the maximal values reached during the episode.
At presentation, for both children and adults, the H-score was more efficient than adapted HLH-2004 guidelines to identify HLH. The diagnostic sensitivity and specificity were respectively 100% and 80% for children and 90% and 79% for adults. However, for adults, performances became comparable between adapted HLH-2004 guidelines and H-score as patient clinical status worsened. The specificity decreased to 73% for the same sensitivity.
The adapted HLH-2004 guidelines seem less powerful and H-score seems to be more appropriate for children, which may be due to less significantly marked biological features. For adults, H-score performances are better when determined at presentation. The cutoff value of the H-score should be adapted depending on the target population to obtain optimal specificity.