The Use of Ferritin to Identify Critically Ill Patients With Secondary Hemophagocytic Lymphohistiocytosis

The Use of Ferritin to Identify Critically Ill Patients With Secondary Hemophagocytic Lymphohistiocytosis
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DOI:
10.1097/ccm.0000000000001878
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发表时间:
2016-11-01
影响因子:
8.8
通讯作者:
Tamion, Fabienne
Tamion, Fabienne
中科院分区:
医学1区
文献类型:
--
作者:
Grange, Steven;Buchonnet, Gerard;Tamion, Fabienne

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目的:血小板减少症是ICU中一种常见的多因素疾病。在ICU的大多数病例中,噬血细胞症是感染性起源的主要解释机制之一,可能与噬血细胞淋巴组织细胞增多综合征相结合。噬血细胞性淋巴组织细胞增多症在ICU中可能未被充分诊断,尽管它与戏剧性的结果相关。这项工作的主要目的是确定继发性噬血细胞性淋巴组织细胞病的频率,以及死亡率的主要预后因素。设计/背景:我们对2000年1月1日至2012年8月22日期间所有疑似或诊断为噬血细胞性淋巴组织细胞病的成年患者进行了回顾性观察研究。患者:共有106例(42%)患者在骨髓检查中出现明显的噬血细胞症,用于探索血小板减少症、双氧体减少症或全血细胞减少症。测量和主要结果:患者年龄中位数为56岁(45-68岁),简化急性生理评分2中位数为55岁(3868岁)。入院的主要原因是血流动力学不稳定(58%),主要与败血症有关(45%)。106例患者中有81例(76%)为细菌感染,其中32例(30%)为大肠杆菌感染。106例患者中46例(43%)在ICU死亡。他们明显年龄较大,具有较高的简化急性生理评分2,血浆乳酸去氢化酶胆红素和血清铁蛋白。与幸存者相比,非幸存者的纤维蛋白原和巨核细胞百分比明显较低。在多变量分析中,只有血清铁蛋白能显著预测与噬血症相关的死亡。血清铁蛋白大于2000 μ g/L预测死亡的敏感性为71%,特异性为76%。巨核细胞百分比的下降也预示着ICU患者的死亡。结论:噬血细胞症在血小板减少的脓毒症患者中很常见,经常包括在感染后噬血细胞淋巴组织细胞增多症中。我们的研究表明,铁蛋白可能是这些患者的可靠预后标志物,并对讨论噬血细胞性淋巴组织细胞增多症的特异性治疗特别感兴趣。
Objectives: Thrombocytopenia is a common, multifactorial, finding in ICU. Hemophagocytosis is one of the main explanatory mechanisms, possibly integrated into hemophagocytic lymphohistiocytosis syndrome, of infectious origin in the majority of cases in ICU. The hemophagocytic lymphohistiocytosis is probably underdiagnosed in the ICU, although it is associated with dramatic outcomes. The main objectives of this work were to identify the frequency of secondary hemophagocytic lymphohistiocytosis, and the main prognostic factors for mortality.Design/Setting: We conducted a retrospective observational study in all adult patients admitted with suspected or diagnosed hemophagocytic lymphohistiocytosis, between January 1, 2000, and August 22, 2012.Patients: A total of 106 patients (42%) had significant hemophagocytosis on bone marrow examination, performed for exploration of thrombocytopenia, bicytopenia, or pancytopenia. Measurements and MainResults: The median age was 56 (45-68) and the median Simplified Acute Physiology Score 2 was 55 (3868). The main reason for ICU admission was hemodynamic instability (58%), predominantly related to sepsis (45% cases). The main precipitating factor found was a bacterial infection in 81 of 106 patients (76%), including 32 (30%) with Escherichia coli infection. Forty six of 106 patients (43%) died in the ICU. They were significantly older, had higher Simplified Acute Physiology Score 2, plasma lactate des-hydrogenase bilirubin, and serum ferritin. The fibrinogen and the percentage of megakaryocytes were significantly lower in nonsurvivors when compared with survivors. In multivariate analysis, only serum ferritin significantly predicted death related to hemophagocytosis. A serum ferritin greater than 2,000 mu g/L predicted death with a sensitivity of 71% and a specificity of 76%. A decreased percentage of megakaryocytes also predicted patient death in the ICU.Conclusions: Hemophagocytosis is common in thrombocytopenic patients with sepsis, frequently included in a postinfectious hemophagocytic lymphohistiocytosis setting. Our study reveals that ferritin could be a reliable prognostic marker in these patients, and hold particular interest in discussing a specific treatment for hemophagocytic lymphohistiocytosis.