Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

作者：Laurence Fardet, Lionel Galicier, Olivier Lambotte, Christophe Marzac, Cédric Aumont, Doumit Chahwan, Paul Coppo, Gilles Hejblum · 发表于：Arthritis & Rheumatology · 年份：2014 · DOI：10.1002/art.38690 · 被引用次数：1501 · 研究领域：Autoimmune and Inflammatory Disorders Research、Fibromyalgia and Chronic Fatigue Syndrome Research、Childhood Cancer Survivors' Quality of Life

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 ...