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Performances of the H-Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adult and Pediatric Patients

2016; Oxford University Press; Volume: 145; Issue: 6 Linguagem: Inglês

10.1093/ajcp/aqw076

1943-7722

France Debaugnies, Bhavna Mahadeb, Alina Ferster, Nathalie Meuleman, Laurence Rozen, Anne Demulder, Francis Corazza,

Family and Disability Support Research

Objectives: 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. Methods: 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. Results: 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. Conclusions: 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.