Department of Paediatrics, Kuo General Hospital, Tainan, Taiwan.
Eur J Haematol. 2012 Nov;89(5):417-22. doi: 10.1111/ejh.12006. Epub 2012 Sep 7.
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal condition in children with Epstein-Barr virus (EBV)-associated infectious mononucleosis (IM). This study aimed to identify commonly available clinical and laboratory predictors that might help clinicians decide to perform the bone marrow and immunological tests for HLH in paediatric EBV-associated IM.
A retrospective case-control study of patients aged <18 yr diagnosed with EBV-associated IM and HLH from 1991 to 2010 in a tertiary medical centre was conducted. A diagnosis of HLH was defined as fulfilling the criteria of the guidelines of the HLH-2004 protocol of the Histiocyte Society and consisted of at least evidence of hemophagocytosis in a bone marrow biopsy.
A total of 177 IM and 27 HLH patients were enrolled. The mean age was 5.3 yr with a female-to-male ratio of 1.06. The most common characteristics (>70% of patients) were fever, lymphadenopathy and hepatomegaly. In addition to the diagnostic criteria of HLH including fever, splenomegaly, cytopenia, hyperferritinaemia, hypertriglyceridemia and/or hypofibrinogenaemia, children with HLH had a significantly higher rate of prolonged fever >10 d, hepatomegaly, jaundice, general malaise, elevated aspartate aminotransferase, lactate dehydrogenase, C-reactive protein and hypoalbuminaemia compared to those with IM (all P < 0.01). Multiple logistic regression confirmed that hypoalbuminaemia (OR = 23.1, P = 0.01) was an independent predictor of paediatric HLH, with a high sensitivity (96%) and a good negative likelihood ratio (0.06) in patients with EBV-associated IM.
Hypoalbuminaemia is a unique characteristic and potentially a valuable predictor for HLH in paediatric EBV-associated IM.
噬血细胞性淋巴组织细胞增生症(HLH)是儿童感染性单核细胞增多症(IM)合并 EBV 感染时一种潜在致命的疾病。本研究旨在确定一些常见的临床和实验室预测指标,以帮助临床医生决定对 EBV 相关 IM 患儿进行骨髓和免疫检查以明确是否合并 HLH。
对 1991 年至 2010 年在一家三级医疗中心诊断为 EBV 相关 IM 合并 HLH 的 <18 岁患者进行回顾性病例对照研究。HLH 的诊断标准为符合噬血细胞性淋巴组织细胞增生症-2004 年(HLH-2004)方案的组织细胞协会指南,并至少在骨髓活检中发现噬血细胞现象。
共纳入 177 例 IM 患者和 27 例 HLH 患者。平均年龄为 5.3 岁,男女比例为 1.06。最常见的特征(>70%的患者)为发热、淋巴结病和肝肿大。除了 HLH 的诊断标准,包括发热、脾肿大、血细胞减少、高铁蛋白血症、高三酰甘油血症和/或低纤维蛋白原血症外,HLH 患儿发热时间>10 d、肝肿大、黄疸、全身不适、天门冬氨酸转氨酶、乳酸脱氢酶、C 反应蛋白和低白蛋白血症的发生率显著高于 IM 患儿(均 P <0.01)。多因素逻辑回归证实低白蛋白血症(OR=23.1,P=0.01)是 EBV 相关 IM 患儿发生 HLH 的独立预测因子,其在 EBV 相关 IM 患儿中的灵敏度为 96%,阴性似然比为 0.06。
低白蛋白血症是 EBV 相关 IM 患儿 HLH 的一个独特特征,可能是一个有价值的预测指标。