France Debaugnies 1 , Bhavna Mahadeb 2 , Alina Ferster 3 , Nathalie Meuleman 4 , Laurence Rozen 5 , Anne Demulder 5 , Francis Corazza 2 Show Affiliations »
Abstract
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. © American Society for Clinical Pathology, 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
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. © American Society for Clinical Pathology, 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
Entities: Disease
Species
Keywords:
Adults; Diagnostic hematology; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; Pediatric
Mesh： See more »
Year: 2016
PMID： 27298397 DOI： 10.1093/ajcp/aqw076
Source DB: PubMed Journal: Am J Clin Pathol ISSN： 0002-9173 Impact factor: 2.493