Batu E D, Erden A, Seyhoğlu E, Kilic L, Büyükasık Y, Karadag O, Bilginer Y, Bilgen S A, Akdogan A, Kiraz S, Ertenli A I, Özen S, Kalyoncu U
a Department of Paediatrics, Division of Rheumatology , Hacettepe University Faculty of Medicine , Ankara , Turkey.
b Department of Internal Medicine, Division of Rheumatology , Hacettepe University Faculty of Medicine , Ankara , Turkey.
Scand J Rheumatol. 2017 Jan;46(1):44-48. doi: 10.3109/03009742.2016.1167951. Epub 2016 Jun 30.
Reactive haemophagocytic syndrome (RHS) is a hyperinflammatory disorder often occurring in the background of several disorders such as infections, malignancies, and rheumatic diseases. Recently, a score known as the HScore was developed for the diagnosis of RHS. In the original study, most of the patients had underlying haematological malignancy or infection and the best cut-off value for the HScore was 169 (sensitivity 93%; specificity 86%). In this study we aimed to analyse the performance of the HScore in rheumatic disease-related RHS.
The patients with rheumatic disorders evaluated in the Departments of Rheumatology and Paediatric Rheumatology at Hacettepe University, Ankara, Turkey between 2002 and 2014 were reviewed retrospectively. The first group (n = 30) consisted of patients with RHS; the control group (n = 64) included patients with active rheumatic diseases without RHS.
In the RHS group, 14 (46.7%) had adult-onset Still's disease (AOSD), 10 (33.3%) systemic juvenile idiopathic arthritis (SJIA), and six (20%) systemic lupus erythematosus (SLE). The control group (n = 64) consisted of 32 (50%) AOSD, 13 (20.3%) SJIA, and 19 (29.7%) SLE patients. Applying the HScore to the RHS patients, the best cut-off value was 190.5 with a sensitivity of 96.7% and specificity of 98.4%. When we excluded the patients from the control group who had not had bone marrow aspiration (n = 23), the same cut-off (190.5) performed best (sensitivity 96.7%; specificity 97.6%). Applying the 2004 haemophagocytic lymphohistiocytosis (HLH-2004) criteria gave a sensitivity of 56.6% and a specificity of 100% in the whole study group.
In our study, a cut-off value for the HScore different from the original study performed better. Further studies are warranted to determine optimum cut-off values in different studies.
反应性噬血细胞综合征（RHS）是一种高炎症性疾病，常发生于多种疾病背景下，如感染、恶性肿瘤和风湿性疾病。最近，一种名为HScore的评分系统被开发用于RHS的诊断。在最初的研究中，大多数患者患有潜在的血液系统恶性肿瘤或感染，HScore的最佳临界值为169（敏感性93%；特异性86%）。在本研究中，我们旨在分析HScore在风湿性疾病相关RHS中的表现。
回顾性分析2002年至2014年期间在土耳其安卡拉哈杰泰佩大学风湿病科和儿科风湿病科评估的风湿性疾病患者。第一组（n = 30）为RHS患者；对照组（n = 64）包括无RHS的活动性风湿性疾病患者。
在RHS组中，14例（46.7%）为成人斯蒂尔病（AOSD），10例（33.3%）为系统性幼年特发性关节炎（SJIA），6例（20%）为系统性红斑狼疮（SLE）。对照组（n = 64）包括32例（50%）AOSD、13例（20.3%）SJIA和19例（29.7%）SLE患者。将HScore应用于RHS患者，最佳临界值为190.5，敏感性为96.7%，特异性为98.4%。当我们排除对照组中未进行骨髓穿刺的患者（n = 23）时，相同的临界值（190.5）表现最佳（敏感性96.7%；特异性97.6%）。在整个研究组中，应用2004年噬血细胞性淋巴组织细胞增生症（HLH - 2004）标准的敏感性为56.6%，特异性为100%。
在我们的研究中，与原始研究不同的HScore临界值表现更好。有必要进行进一步研究以确定不同研究中的最佳临界值。
