Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome
Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome
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DOI：
10.1002/art.38690
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发表时间：
2014-09-01
影响因子：
13.3
通讯作者：
Hejblum, Gilles
中科院分区：
文献类型：
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作者：
Fardet, Laurence;Galicier, Lionel;Hejblum, Gilles
Objective.因为它没有独特的临床、生物学或组织学特征，反应性噬血细胞综合征可能难以与其他疾病（如严重脓毒症或血液恶性肿瘤）区分。本研究旨在开发和验证反应性噬血细胞综合征的诊断评分。一个由312名患者组成的多中心回顾性队列，这些患者被专家判定为患有反应性噬血细胞综合征（n = 162），被专家判定为没有反应性噬血细胞综合征（n = 104），或其中反应性噬血细胞综合征的诊断未确定（n = 46），用于构建和验证反应性噬血细胞综合征诊断评分，称为HScore。评价了10个解释变量与噬血细胞综合征诊断的相关性，并使用逻辑回归计算评分中包含的每个标准的权重。使用开发和验证数据集评估评分的性能。9个变量（3个临床[即，已知潜在的免疫抑制、高温、器官肿大]，5生物学[即，甘油三酯、铁蛋白、血清谷草转氨酶和纤维蛋白原水平、血细胞减少]，和1例细胞学[即，骨髓抽吸物上的噬血细胞特征]）保留在HScore中。分配给每个变量的可能点数范围为0-18（已知潜在免疫抑制）和0-64（甘油三酯水平）。反应性噬血细胞综合征阳性诊断患者的中位HS评分为230（四分位距[IQR] 203-257），阴性诊断患者的中位HS评分为125（IQR 91-150）。发生噬血细胞综合征的概率为< 1% with an HScore of 99% with an HScore of >= 250。HScore可用于评估个体患反应性噬血细胞综合征的风险。
Objective. Because it has no unique clinical, biologic, or histologic features, reactive hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis or hematologic malignancies. This study was undertaken to develop and validate a diagnostic score for reactive hemophagocytic syndrome.Methods. A multicenter retrospective cohort of 312 patients who were judged by experts to have reactive hemophagocytic syndrome (n = 162), were judged by experts to not have reactive hemophagocytic syndrome (n = 104), or in whom the diagnosis of reactive hemophagocytic syndrome was undetermined (n = 46) was used to construct and validate the reactive hemophagocytic syndrome diagnostic score, called the HScore. Ten explanatory variables were evaluated for their association with the diagnosis of hemophagocytic syndrome, and logistic regression was used to calculate the weight of each criterion included in the score. Performance of the score was assessed using developmental and validation data sets.Results. Nine variables (3 clinical [i.e., known underlying immunosuppression, high temperature, organomegaly], 5 biologic [i.e., triglyceride, ferritin, serum glutamic oxaloacetic transaminase, and fibrinogen levels, cytopenia], and 1 cytologic [i.e., hemophagocytosis features on bone marrow aspirate]) were retained in the HScore. The possible number of points assigned to each variable ranged from 0-18 for known underlying immunosuppression to 0-64 for triglyceride level. The median HScore was 230 (interquartile range [IQR] 203-257) for patients with a positive diagnosis of reactive hemophagocytic syndrome and 125 (IQR 91-150) for patients with a negative diagnosis. The probability of having hemophagocytic syndrome ranged from < 1% with an HScore of 99% with an HScore of >= 250.Conclusion. The HScore can be used to estimate an individual's risk of having reactive hemophagocytic syndrome.