Am J Hematol. 2010 Sep;85(9):719-22. doi: 10.1002/ajh.21795.
We assessed the clinical significance of T or B cell clonality and Epstein-Barr virus (EBV) infection in adult patients with hemophagocytic lymphohistiocytosis (HLH) to identify factors related to prognosis. A total of 30 adult patients with diagnosed HLH were included in the study. In all patients, EBV-DNA in peripheral blood was examined by quantitative real-time polymerase chain reaction and bone marrow cells were examined for clonal rearrangement of T cell receptor gamma(TCRG) and immunoglobulin heavy chain (IGH) genes. TCRG clones were detected in 10 patients (33.3%) and IGH clones were detected in 8 patients (26.7%). We found no correlation between clonality and patient outcome. The patients less than 1,000 copies (mL)21 of EBVDNA showed a significantly higher clinical response (P 5 0.008) and longer overall survival (P 5 0.01) than those with high viral load of EBV-DNA. Our results suggest that TCRG and IGH rearrangement do not have any clinical significance in adult patients with HLH, but that high viral load of EBV-DNA may be a risk factor for poor outcomes. In HLH, high viral load of EBV-DNA should thus suggest a prompt approach with aggressive therapeutic interventions.
我们评估了 T 或 B 细胞克隆性和 Epstein-Barr 病毒 (EBV) 感染在成人噬血细胞性淋巴组织细胞增生症 (HLH) 患者中的临床意义，以确定与预后相关的因素。本研究共纳入 30 例确诊为 HLH 的成年患者。所有患者均通过实时定量聚合酶链反应检测外周血 EBV-DNA，并检测骨髓细胞 T 细胞受体γ (TCRG) 和免疫球蛋白重链 (IGH) 基因的克隆性重排。10 例患者（33.3%）检测到 TCRG 克隆，8 例患者（26.7%）检测到 IGH 克隆。我们发现克隆性与患者预后之间无相关性。EBV-DNA 拷贝数低于 1,000 拷贝/毫升的患者临床反应显著更高（P = 0.008），总生存期更长（P = 0.01）。我们的结果表明，TCRG 和 IGH 重排在成人 HLH 患者中没有任何临床意义，但 EBV-DNA 的高病毒载量可能是预后不良的危险因素。因此，在 HLH 中，高病毒载量的 EBV-DNA 应提示采用积极治疗干预的方法。
