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Diagnostic and prognostic value of low percentage of glycosylated ferritin in acquired hemophagocytic lymphohistiocytosis: A single‐center study
Author(s) -
Nabergoj M.,
Marinova M.,
Binotto G.,
Brugnaro L.,
Zaninotto M.,
Plebani M.,
Semenzato G.,
Vianello F.
Publication year - 2017
Publication title -
international journal of laboratory hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.705
H-Index - 55
eISSN - 1751-553X
pISSN - 1751-5521
DOI - 10.1111/ijlh.12713
Subject(s) - hemophagocytic lymphohistiocytosis , hemophagocytosis , ferritin , medicine , gastroenterology , bone marrow , immunology , disease , pancytopenia
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.