Department of Anesthesiology and Operative Intensive Care Medicine (CCM, CVK), Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Augustenburger Platz 1, D-13353 Berlin, Germany.
Berlin Institute of Health (BIH), Berlin, Germany.
Crit Care Med. 2020 Apr;48(4):459-465. doi: 10.1097/CCM.0000000000004131.
Hyperferritinemia is frequently seen in critically ill patients. A rather rare though life-threatening condition related to severely elevated ferritin is hemophagocytic lymphohistiocytosis. We analyze ferritin levels to differentiate hemophagocytic lymphohistiocytosis from other causes of hyperferritinemia in a mixed cohort of critically ill patients.
Retrospective observational study.
Adult surgical, anesthesiologic, and medical ICUs of a university hospital.
Critical care patients (≥ 18 yr old) admitted to any of the adult ICUs at Charité - Universitätsmedizin Berlin between January 2006 and August 2018 with at least one ferritin value and hyperferritinemia (≥ 500 µg/L).
None.
Patients were categorized into hemophagocytic lymphohistiocytosis, sepsis, septic shock, and other diagnoses. These were further categorized into 17 subgroups. Hemophagocytic lymphohistiocytosis diagnosis was based on Hemophagocytic Lymphohistiocytosis-2004 criteria and the HScore. Of 2,623 patients with hyperferritinemia, 40 were considered to have hemophagocytic lymphohistiocytosis (1.52%). Maximum ferritin levels were highest in hemophagocytic lymphohistiocytosis patients compared with all other disease groups (each p < 0.001). Sepsis and septic shock patients had higher maximum ferritin levels than patients with other diagnoses (each p < 0.001). A maximum ferritin value of 9,083 µg/L was at 92.5% sensitivity and 91.9% specificity for hemophagocytic lymphohistiocytosis (area under the curve, 0.963; 95% CI, 0.949-0.978). Of all subgroups with other diagnoses, maximum ferritin levels were highest in patients with varicella-zoster virus, hepatitis, or malaria (median, 1,935, 1,928, and 1,587 µg/L, respectively). Maximum ferritin levels were associated with increased in-hospital mortality (odds ratio, 1.518 per log µg/L [95% CI, 1.384-1.665 per log µg/L]; p < 0.001).
This is the largest study of patients with ferritin available in a mixed ICU cohort. Ferritin levels in patients with hemophagocytic lymphohistiocytosis, sepsis, septic shock, and other conditions were distinctly different, with the highest ferritin levels observed in hemophagocytic lymphohistiocytosis patients. Maximum ferritin of 9,083 µg/L showed high sensitivity and specificity and, therefore, may contribute to improved diagnosis of hemophagocytic lymphohistiocytosis in ICU. The inclusion of ferritin into the sepsis laboratory panel is warranted.
铁蛋白升高在危重症患者中较为常见。噬血细胞性淋巴组织细胞增生症是一种与铁蛋白严重升高相关的罕见但危及生命的疾病。我们分析铁蛋白水平，以在重症监护患者的混合队列中区分噬血细胞性淋巴组织细胞增生症与其他原因引起的铁蛋白升高。
回顾性观察性研究。
柏林夏里特大学附属医院成人外科、麻醉和内科重症监护病房。
2006 年 1 月至 2018 年 8 月期间入住柏林夏里特大学附属医院成人 ICU 的重症监护患者（≥ 18 岁），至少有一次铁蛋白值和铁蛋白升高（≥ 500μg/L）。
无。
患者分为噬血细胞性淋巴组织细胞增生症、脓毒症、脓毒性休克和其他诊断。这些进一步分为 17 个亚组。噬血细胞性淋巴组织细胞增生症的诊断基于噬血细胞性淋巴组织细胞增生症-2004 标准和 H 评分。在 2623 例铁蛋白升高的患者中，40 例被认为患有噬血细胞性淋巴组织细胞增生症（1.52%）。噬血细胞性淋巴组织细胞增生症患者的最大铁蛋白水平与所有其他疾病组相比最高（p 均<0.001）。脓毒症和脓毒性休克患者的最大铁蛋白水平高于其他诊断患者（p 均<0.001）。最大铁蛋白值为 9083μg/L 时，对噬血细胞性淋巴组织细胞增生症的敏感性为 92.5%，特异性为 91.9%（曲线下面积，0.963；95%置信区间，0.949-0.978）。在其他诊断的所有亚组中，水痘-带状疱疹病毒、肝炎或疟疾患者的最大铁蛋白水平最高（中位数分别为 1935μg/L、1928μg/L 和 1587μg/L）。最大铁蛋白水平与住院期间死亡率增加相关（优势比，每 logμg/L 增加 1.518[95%置信区间，每 logμg/L 增加 1.384-1.665]；p<0.001）。
这是在混合 ICU 队列中对铁蛋白进行的最大规模研究。噬血细胞性淋巴组织细胞增生症、脓毒症、脓毒性休克和其他情况下患者的铁蛋白水平明显不同，噬血细胞性淋巴组织细胞增生症患者的铁蛋白水平最高。最大铁蛋白值为 9083μg/L 时具有较高的敏感性和特异性，因此可能有助于提高 ICU 中噬血细胞性淋巴组织细胞增生症的诊断。将铁蛋白纳入脓毒症实验室检测面板是合理的。
