Imashuku S, Hibi S, Tabata Y, Sako M, Sekine Y, Hirayama K, Sakazaki H, Maeda N, Kito H, Shichino H, Mugishima H
Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan.
Med Pediatr Oncol. 1998 Sep;31(3):131-7. doi: 10.1002/(sici)1096-911x(199809)31:3<131::aid-mpo1>3.0.co;2-c.
Viruses may induce primary as well as secondary hemophagocytic lymphohistiocytosis (HLH), but it may not be possible to discriminate between these two in patients with a negative family history. Among these HLH cases, fulminant and fatal virus-associated hemophagocytic syndrome (VAHS) occurs mostly in relation to Epstein-Barr virus (EBV) infection. Although the immunological characteristics of EB-VAHS were previously reported, data on non-EB-VAHS were sporadic and fragmentary. This study has compared the clearly distinguishable groups of EBV-positive vs. EBV-negative HLH cases.
Among 26 patients with EBV-related HLH and 12 patients with non-EBV HLH, peripheral blood mononuclear cell (PBMC) subsets and serum concentrations of cytokines at the active phase of the disease were compared. Blood and bone marrow smears were also compared.
The frequency of the CD3+HLADR+ subset in PBMC (median 34.3% vs. 4.8%), of serum concentrations of interferon (IFN)-gamma (median 105 U/ml vs. 2.4 U/ml), and of soluble interleukin-2-receptor (sIL-2R) (median 14,700 U/ml vs. 3,412 U/ml) were significantly different between these two groups. Morphological characteristics were noted for EBV-related HLH cases. Mortality also differed between these two groups, 9/26 vs. 0/12 (P = 0.05). Data indicate pronounced immunological imbalance and poor prognosis in EBV-related HLH cases. These parameters could be useful for determining an EBV involvement as well as risk factors in the early care and treatment of HLH patients.
病毒可诱发原发性及继发性噬血细胞性淋巴组织细胞增生症（HLH），但对于家族史阴性的患者，可能无法区分这两种情况。在这些HLH病例中，暴发性和致命性病毒相关噬血细胞综合征（VAHS）大多与爱泼斯坦-巴尔病毒（EBV）感染有关。尽管先前已报道了EB-VAHS的免疫学特征，但关于非EB-VAHS的数据零散且不完整。本研究比较了EBV阳性与EBV阴性HLH病例这两个明显可区分的组。
在26例EBV相关HLH患者和12例非EBV HLH患者中，比较了疾病活动期外周血单个核细胞（PBMC）亚群和细胞因子的血清浓度。还比较了血液和骨髓涂片。
两组之间PBMC中CD3 + HLADR +亚群的频率（中位数34.3%对4.8%）、干扰素（IFN）-γ的血清浓度（中位数105 U/ml对2.4 U/ml）以及可溶性白细胞介素-2受体（sIL-2R）（中位数14,700 U/ml对3,412 U/ml）存在显著差异。EBV相关HLH病例有形态学特征。两组的死亡率也不同，分别为9/26对0/12（P = 0.05）。数据表明EBV相关HLH病例存在明显的免疫失衡和不良预后。这些参数可有助于确定EBV的参与情况以及HLH患者早期护理和治疗中的危险因素。
