title: Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis.
abstract: Hyperferritinemia is a hallmark of hemophagocytic lymphohistiocytosis (HLH). In the HLH-2004 criteria, a ferritin value >500 µg/L is considered positive. However, this level was not determined based on evidence. We compared 123 patients with HLH and 320 patients with other hyperferritinemic conditions, calculated a receiver-operating characteristic, and determined sensitivity and specificity for different values. At 2,000 µg/L a trade-off is reached with sensitivity at 70% and specificity at 68%. If familial HLH and virus-associated acquired HLH are analyzed separately, sensitivity and specificity are similar for this level. The results may guide a potential modification of the current HLH criteria.
authors.affiliation: Pediatric Hematology and Oncology, University Hospital Medical Center, Hamburg, Germany.
authors.forename: Kai
authors.initials: K
authors.lastname: Lehmberg
authors.forename: Kenneth L
authors.initials: KL
authors.lastname: McClain
authors.forename: Gritta E
authors.initials: GE
authors.lastname: Janka
authors.forename: Carl E
authors.initials: CE
authors.lastname: Allen
mesh_terms: D005293:Ferritins / Q000097:blood*; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000175:diagnosis*; D012372:ROC Curve
chemical_list: D005293:Ferritins
keywords: HLH; diagnostic criteria; ferritin; hemophagocytic lymphohistiocytosis; hyperinflammation; macrophage activation syndrome