Hejblum Gilles, Lambotte Olivier, Galicier Lionel, Coppo Paul, Marzac Christophe, Aumont Cédric, Fardet Laurence
INSERM, UMR_S 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, Paris, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, Paris, France; AP-HP, Hôpital Saint-Antoine, Unité de Santé Publique, Paris, France.
AP-HP, Hôpital de Bicêtre, Service de Médecine Interne, Le Kremlin Bicêtre, France; Université Paris-Sud 11, Faculté de Médecine Paris-Sud 11, Le Kremlin Bicêtre, France.
PLoS One. 2014 Apr 7;9(4):e94024. doi: 10.1371/journal.pone.0094024. eCollection 2014.
The diagnosis of the reactive form of hemophagocytic syndrome in adults remains particularly difficult since none of the clinical or laboratory manifestations are specific. We undertook a study in order to elicit which features constitute helpful criteria for a positive diagnosis. In this Delphi study, the features investigated in the questionnaire and the experts invited to participate in the survey were issued from a bibliographic search. The questionnaire was iteratively proposed to experts via a web-based application with a feedback of the results observed at the preceding Delphi round. Experts were asked to label each investigated criterion in one of the following categories: absolutely required, important, of minor interest, or not assessable in the routine practice environment. A positive consensus was a priori defined as at least 75% answers observed in the categories absolutely required and important. The questionnaire investigated 26 criteria and 24 experts originating from 13 countries participated in the second and final Delphi round. A positive consensus was reached for the nine following criteria: unilineage cytopenia, bicytopenia, pancytopenia, presence of hemophagocytosis pictures on a bone marrow aspirate or on a tissue biopsy, high ferritin level, fever, organomegaly, presence of a predisposing underlying disease, and high level of lactate dehydrogenase. A negative consensus was reached for 13 criteria, and an absence of consensus was observed for 4 criteria. The study constitutes the first initiative to date for defining international guidelines devoted to the positive diagnosis of the reactive form of hemophagocytic syndrome.
成人反应性噬血细胞综合征的诊断仍然特别困难，因为临床或实验室表现均无特异性。我们开展了一项研究，以确定哪些特征构成阳性诊断的有用标准。在这项德尔菲研究中，问卷中调查的特征以及受邀参与调查的专家均来自文献检索。问卷通过基于网络的应用程序反复向专家提出，并反馈上一轮德尔菲调查中观察到的结果。要求专家将每个调查标准标记为以下类别之一：绝对必要、重要、次要兴趣或在常规实践环境中不可评估。预先将阳性共识定义为在绝对必要和重要类别中观察到至少75%的答案。问卷调查了26项标准，来自13个国家的24名专家参与了第二轮也是最后一轮德尔菲调查。就以下九项标准达成了阳性共识：单系血细胞减少、双系血细胞减少、全血细胞减少、骨髓穿刺或组织活检中存在噬血细胞图像、铁蛋白水平高、发热、器官肿大、存在易患基础疾病以及乳酸脱氢酶水平高。13项标准达成了阴性共识，4项标准未达成共识。该研究是迄今为止确定反应性噬血细胞综合征阳性诊断国际指南的首个举措。
