Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xuan Wu Qu, 100050, Beijing, People's Republic of China.
Int J Hematol. 2009 Nov;90(4):501-505. doi: 10.1007/s12185-009-0391-5. Epub 2009 Aug 5.
Elevated levels of serum ferritin and a low percentage of glycosylated ferritin have been reported in adult-onset Still's disease (AOSD) as well as in hemophagocytic lymphohistiocytosis (HLH). The objective of the current study was to investigate total ferritin levels and the percentage of glycosylated ferritin in patients with secondary HLH. From October 2007 to October 2008, 29 patients with suspected HLH older than 14 years of age treated at Beijing Friendship Hospital were enrolled. Twenty-five healthy volunteers were recruited as controls. The suspected HLH patients were further divided into secondary HLH-confirmed (confirmed) (22 out of 29) and HLH-unconfirmed (unconfirmed) (7 out of 29) groups based on HLH-2004 diagnostic criteria. Total serum ferritin levels and the percentage of glycosylated ferritin were determined in subjects at the time of admission. Significantly higher levels of total serum ferritin were observed in confirmed (2,897.6 +/- 1,837.2 microg/L) compared with unconfirmed (653.1 +/- 249.1 microg/L) patients (P < 0.01) or controls (414.6 +/- 212.6 microg/L) (P < 0.01). A significantly lower percentage of glycosylated ferritin was observed in the confirmed (20.5 +/- 10.1%) compared with the unconfirmed (48.7 +/- 12.1%) group (P < 0.01) or the control group (53.6 +/- 13.3%) (P < 0.01). In addition, a low percentage of glycosylated ferritin was observed in HLH patients. Finally, regarding the diagnosis of HLH based on a low percentage of glycosylated ferritin, the sensitivity and specificity, as well as positive and negative predictive values were 0.86, 0.71, 0.91, and 0.63, respectively. For the diagnosis of HLH based on hyperferritinemia, the sensitivity, specificity, positive, and negative predictive values were 0.82, 0.43, 0.82, and 0.43, respectively. The results of this study suggest that a low percentage of glycosylated ferritin is associated with HLH. On comparison with hyperferritinemia, a low percentage of glycosylated ferritin appeared to be more specific, sensitive, and predictive of HLH. In conclusion, a low percentage of glycosylated ferritin may be a useful marker for the early diagnosis of secondary HLH.
血清铁蛋白水平升高和糖化铁蛋白比例降低已在成人斯蒂尔病（AOSD）和噬血细胞性淋巴组织细胞增多症（HLH）中报道。本研究的目的是研究继发性 HLH 患者的总铁蛋白水平和糖化铁蛋白比例。2007 年 10 月至 2008 年 10 月，北京友谊医院收治了 29 例年龄大于 14 岁的疑似 HLH 患者。招募了 25 名健康志愿者作为对照。根据 HLH-2004 诊断标准，将疑似 HLH 患者进一步分为继发性 HLH 确诊（确诊）（29 例中的 22 例）和 HLH 未确诊（未确诊）（29 例中的 7 例）组。在入院时测定受试者的血清总铁蛋白水平和糖化铁蛋白比例。确诊组（2897.6±1837.2μg/L）患者的总血清铁蛋白水平明显高于未确诊组（653.1±249.1μg/L）（P<0.01）或对照组（414.6±212.6μg/L）（P<0.01）。确诊组（20.5±10.1%）的糖化铁蛋白比例明显低于未确诊组（48.7±12.1%）（P<0.01）或对照组（53.6±13.3%）（P<0.01）。此外，HLH 患者的糖化铁蛋白比例较低。最后，基于低糖化铁蛋白比例诊断 HLH 的敏感性、特异性、阳性和阴性预测值分别为 0.86、0.71、0.91 和 0.63。基于高血铁蛋白诊断 HLH 的敏感性、特异性、阳性和阴性预测值分别为 0.82、0.43、0.82 和 0.43。本研究结果表明，低糖化铁蛋白比例与 HLH 有关。与高血铁蛋白相比，低糖化铁蛋白比例似乎更特异、敏感、预测 HLH。总之，低糖化铁蛋白比例可能是继发性 HLH 早期诊断的有用标志物。
