Nabergoj M, Marinova M, Binotto G, Brugnaro L, Zaninotto M, Plebani M, Semenzato G, Vianello F
Department of Medicine, Hematology and Clinical Immunology Unit, University of Padova, Padova, Italy.
Department of Medicine, Laboratory Medicine Unit, University of Padova, Padova, Italy.
Int J Lab Hematol. 2017 Dec;39(6):620-624. doi: 10.1111/ijlh.12713. Epub 2017 Jul 18.
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.
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.
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.
Fraction of GF is useful to identify subjects at high risk for early death and therefore in need of early treatment.
噬血细胞性淋巴组织细胞增生症（HLH）是一种具有侵袭性且危及生命的综合征，其特征为过度的免疫激活。糖基化铁蛋白（GF）水平被认为是HLH的高度特异性指标。
我们根据HLH - 04试验标准研究了12例HLH患者以及11例临床和实验室检查怀疑为HLH的患者。GF的百分比通过内部检测方法进行测量。
两组之间唯一有显著差异的生物标志物是GF分数（P <.001）和骨髓中噬血细胞的存在情况（P =.006）。HLH患者的GF百分比显著低于模拟HLH的其他炎症性疾病患者。GF分数≤20%与HLH诊断高度一致。
GF分数有助于识别早期死亡风险高且因此需要早期治疗的患者。
