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Am J Clin Pathol. 2016 Jun;145(6):862-70. doi: 10.1093/ajcp/aqw076. Epub 2016 Jun 12.

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

Author information

1
From the Laboratory of Immunology Laboratory of Biochemistry, Laboratoire National de Santé, Dudelange, Luxembourg. fdebaugn@ulb.ac.be.
2
From the Laboratory of Immunology.
3
Department of Hematology and Oncology, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium.
4
Department of Hematology, Jules Bordet Institute, Brussel, Belgium.
5
Laboratory of Hematology and Hemostasis, Université Libre de Bruxelles, CHU Brugmann, Brussels, Belgium.

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.

KEYWORDS:

Adults; Diagnostic hematology; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; Pediatric

PMID:
27298397
DOI:
10.1093/ajcp/aqw076
[Indexed for MEDLINE]

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