Diagnostic and prognostic value of low percentage of glycosylated ferritin in acquired hemophagocytic lymphohistiocytosis: A single-center study

Int J Lab Hematol. 2017 Dec;39(6):620-624. doi: 10.1111/ijlh.12713. Epub 2017 Jul 18.

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome characterized by an excessive immune activation. Glycosylated ferritin (GF) level has been proposed as highly specific of HLH.

Methods: We have studied 12 subjects with HLH according to the HLH-04 trial criteria and 11 patients with a clinical and laboratoristic suspicion of HLH. The percentage of GF was measured by an in-house assay.

Results: The only biomarkers that were significantly different in the two groups were fraction of GF (P<.001) and the presence of hemophagocytosis in bone marrow (P=.006). Subjects with HLH had significantly lower percentage of GF than patients with other inflammatory conditions mimicking HLH. A fraction of GF ≤20% was strongly consistent with a diagnosis of HLH.

Conclusions: Fraction of GF is useful to identify subjects at high risk for early death and therefore in need of early treatment.

Keywords: differential diagnosis of hyperferritin; glycosylated ferritin; hemophagocytic lymphohistiocytosis.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Aged
  • Female
  • Ferritins / blood*
  • Glycosylation
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / blood*
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Male
  • Middle Aged

Substances

  • Ferritins