Department of Laboratory Medicine and Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Ann Lab Med. 2019 Sep;39(5):430-437. doi: 10.3343/alm.2019.39.5.430.
T cell immunophenotypes in patients with hemophagocytic lymphohistiocytosis (HLH) have been described. Downregulation of CD5 or CD7 on T cells has been reported in patients with Epstein-Barr virus (EBV)-positive HLH. As the utility of T cell immunophenotypes as an adjunctive diagnostic or a prognostic marker for HLH has not been evaluated, we analyzed T cell immunophenotypes in HLH patients for this purpose.
We classified 45 HLH patients into three subgroups: EBV-positive HLH (N=27), EBV-negative secondary HLH (N=15), and familial HLH (N=3). We retrospectively characterized downregulation patterns of CD5 or CD7 on activated T cells, using flow cytometry. Overall survival was estimated using Kaplan-Meier curves and compared using the log-rank test.
An aberrant immunophenotype, including CD5 and/or CD7 downregulation on T cells, was observed in 55.6% (15/27) of the EBV-positive HLH patients and 100% of the familial HLH (3/3). Only one (1/15, 6.7%) patient with EBV-negative secondary HLH showed an aberrant loss of CD7 antigen on CD8 T cells. The presence of an aberrant T cell immunophenotype was not related to overall survival in EBV-positive HLH and EBV-negative secondary HLH patients.
An aberrant T cell immunophenotype may assist in discriminating EBV-negative secondary HLH and EBV-positive HLH. However, it may not be useful as a prognostic marker.
噬血细胞性淋巴组织细胞增生症（HLH）患者的 T 细胞免疫表型已有描述。EBV 阳性 HLH 患者的 T 细胞上 CD5 或 CD7 的下调已被报道。由于 T 细胞免疫表型作为 HLH 的辅助诊断或预后标志物的实用性尚未得到评估，因此我们为此目的分析了 HLH 患者的 T 细胞免疫表型。
我们将 45 例 HLH 患者分为三组：EBV 阳性 HLH（N=27）、EBV 阴性继发性 HLH（N=15）和家族性 HLH（N=3）。我们使用流式细胞术回顾性地描述了活化 T 细胞上 CD5 或 CD7 下调的模式。使用 Kaplan-Meier 曲线估计总生存率，并使用对数秩检验进行比较。
在 55.6%（27/45）的 EBV 阳性 HLH 患者和 100%（3/3）的家族性 HLH 患者中观察到异常免疫表型，包括 T 细胞上 CD5 和/或 CD7 下调。只有 1 例（1/15，6.7%）EBV 阴性继发性 HLH 患者的 CD8 T 细胞上出现 CD7 抗原异常缺失。异常 T 细胞免疫表型的存在与 EBV 阳性 HLH 和 EBV 阴性继发性 HLH 患者的总生存率无关。
异常 T 细胞免疫表型可能有助于区分 EBV 阴性继发性 HLH 和 EBV 阳性 HLH。然而，它可能不是一个有用的预后标志物。
