Medical ICU, Saint-Louis University Hospital, AP-HP, Paris, France.
Faculté de Médecine, Université Paris 7, Paris-Diderot, France.
Crit Care Med. 2021 Sep 1;49(9):e874-e879. doi: 10.1097/CCM.0000000000005038.
To assess whether critically ill hematologic patients without diagnosis of hemophagocytic lymphohistiocytosis may have features mimicking hemophagocytic lymphohistiocytosis according to both diagnostic scores.
A retrospective case-control study.
Hemophagocytic syndrome diagnosis was standardized and based on a consensual diagnosis by at least two experts of a university hospital which is a reference center for hemophagocytic syndrome.
Cases (hemophagocytic syndrome+) consisted in a group of consecutive patients (n = 150) admitted in our ICU between 2007 and 2018. Control group (hemophagocytic syndrome-) consisted in patients included in a prospective multicenter cohort of hematologic patients in whom three independent experts ruled out the diagnosis of hemophagocytic syndrome (n = 1011).
Overall, 1,161 patients were included. Hospital mortality was 45.8% in hemophagocytic syndrome- patients (n = 66) and 38.8% in control patients (n = 392; p = 0.126). Median HScore was 235 (205-262) in hemophagocytic syndrome+ and 42 (18-62) in hemophagocytic syndrome- patients (p < 0.001); number of hemophagocytic lymphohistiocytosis criteria was 4 (4-5) vs 1 (0-1), respectively (p < 0.001). Diagnostic performances of both scores were excellent with area under receiver operating characteristic curve of 0.99 (95% CI, 0.99-0.99) and 0.99 (95% CI, 0.99-0.99) for hemophagocytic lymphohistiocytosis and HScore, respectively. After propensity score matching (n = 144 × 2), the median HScore was 234 (205-262) in hemophagocytic syndrome+ patients versus 49 (18-71) in hemophagocytic syndrome- patients (p < 0.001). Median number of hemophagocytic lymphohistiocytosis criteria was 4 (4-5) in hemophagocytic syndrome+ and 1 (0-1) in hemophagocytic syndrome- patients (p < 0.001). Area under receiver operating characteristic curve was then of 0.98 (95% CI, 0.96-0.99) for hemophagocytic lymphohistiocytosis criteria and 0.99 (95% CI, 0.99-1) for HScore.
In ICU patients, several conditions share some similarities with hemophagocytic syndrome, explaining the poor predictive value of isolated biological markers such as ferritin level. Despite these potential confounding factors, our study suggests HScore and hemophagocytic lymphohistiocytosis criteria to be highly discriminant identifying hemophagocytic syndrome in critically ill patients.
评估未诊断噬血细胞性淋巴组织细胞增生症的危重症血液病患者是否可能根据两种诊断评分标准出现类似噬血细胞性淋巴组织细胞增生症的特征。
回顾性病例对照研究。
噬血细胞综合征的诊断是标准化的，并基于至少两名专家对大学医院的共识诊断，该医院是噬血细胞综合征的参考中心。
病例组（噬血细胞综合征+）由 2007 年至 2018 年期间我院 ICU 连续收治的一组患者（n=150）组成。对照组（噬血细胞综合征-）由纳入一项前瞻性多中心血液病患者队列研究的患者组成，该研究中有三名独立专家排除了噬血细胞综合征的诊断（n=1011）。
共有 1161 名患者纳入研究。噬血细胞综合征-患者的院内死亡率为 45.8%（n=66），对照组患者的死亡率为 38.8%（n=392；p=0.126）。噬血细胞综合征+患者的 H 评分中位数为 235（205-262），噬血细胞综合征-患者的 H 评分中位数为 42（18-62）（p<0.001）；噬血细胞性淋巴组织细胞增生症标准数分别为 4（4-5）和 1（0-1）（p<0.001）。两种评分的诊断性能均极佳，噬血细胞性淋巴组织细胞增生症的受试者工作特征曲线下面积分别为 0.99（95%CI，0.99-0.99）和 0.99（95%CI，0.99-0.99），H 评分分别为 0.99（95%CI，0.99-0.99）。经倾向评分匹配（n=144×2）后，噬血细胞综合征+患者的 H 评分中位数为 234（205-262），噬血细胞综合征-患者的 H 评分中位数为 49（18-71）（p<0.001）。噬血细胞综合征+患者的噬血细胞性淋巴组织细胞增生症标准数中位数为 4（4-5），噬血细胞综合征-患者的标准数中位数为 1（0-1）（p<0.001）。受试者工作特征曲线下面积为噬血细胞性淋巴组织细胞增生症标准 0.98（95%CI，0.96-0.99），H 评分 0.99（95%CI，0.99-1）。
在 ICU 患者中，一些情况与噬血细胞综合征存在一些相似之处，这解释了铁蛋白等孤立的生物学标志物预测能力不佳的原因。尽管存在这些潜在的混杂因素，但我们的研究表明 H 评分和噬血细胞性淋巴组织细胞增生症标准在识别危重症患者中的噬血细胞综合征方面具有高度的判别能力。
