Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis
Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis
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DOI：
10.1002/pbc.21423
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发表时间：
2008-06-01
影响因子：
3.2
通讯作者：
McClain, Kenneth L.
中科院分区：
文献类型：
--
作者：
Allen, Carl E.;Yu, Xiaoying;McClain, Kenneth L.
背景噬血细胞性淋巴组织细胞增生症（HLH）是一种以病理性炎症为特征的潜在致死性疾病。HLH的诊断标准包括发热、脾肿大、血细胞减少、高胆红素血症、低纤维蛋白原血症、异常自然杀伤细胞（NK细胞）功能测定、可溶性IL-2 R α水平升高和铁蛋白水平升高（>500 μ g/L）。这些危重患者的及时治疗可能会因难以确定诊断而延迟。NK细胞功能测定和可溶性IL-2 R α水平可能需要发送到专业实验室。然而，铁蛋白水平是在同一天的基础上，在大多数机构。在这项研究中，我们检测了定量铁蛋白水平在诊断HLH中的效用。Procedure.确定了2003年1月10日至2005年1月10日期间在德克萨斯儿童医院获得的铁蛋白值>500 μ g/L的所有患者。审查患者病历中的铁蛋白水平和住院病程。结果在研究期间，330例患者的铁蛋白水平>500 μ g/L。330例患者中有10例被诊断为HLH。铁蛋白水平超过10，000 μ g/L对HLH的敏感性为90%，特异性为96%。铁蛋白水平显著升高的另一个诊断类别是原因不明的疾病（n = 10），其中只有2例患者进行了全面的HLH评估。结论.铁蛋白水平高于10，000 μ g/L似乎对HLH具有特异性和敏感性。在没有明显病史和新发发热性疾病伴铁蛋白水平高度升高的患者中，应评估HLH的诊断。
Background. Hemophagocytic lymphohistiocytosis (HLH) is a potentially lethal condition characterized by a pathologic inflammation. The diagnostic criteria for HLH include fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, abnormal natural killer cell (NK cell) functional assay, elevated soluble IL-2R alpha level, and elevated ferritin level (>500 mu g/L). Institution of timely therapy in these critically ill patients may be delayed by difficulties establishing the diagnosis. NK cell functional assay and Soluble IL-2R alpha level may require send-out to a specialized lab. However, ferritin level is available on a same-day basis at most institutions. in this study, we examined the utility of quantitative ferritin levels in diagnosing HLH. Procedure. All patients with ferritin Values >500 mu g/L obtained at Texas Children's Hospital between January 10, 2003 and January 10, 2005 were identified. Patient charts were reviewed for ferritin levels and hospital course. Results. During the study interval, 330 patients had ferritin levels >500 mu g/L. Ten of the 330 patients were diagnosed with HLH. A ferritin level over 10,000 mu g/L was 90% sensitive and 96% specific for HLH. Another diagnostic category with significantly elevated ferritin level was illness of unknown cause (n = 10), and only two of these patients were fully evaluated for HLH. Conclusions. Ferritin levels above 10,000 mu g/L appear to be specific and sensitive for HLH. In patients without a significant medical history and a new onset of febrile illness with highly elevated ferritin levels, the diagnosis of HLH should be evaluated.