Lymphocytes In Autoimmune Lymphoproliferative Syndrome

根据扩大的双阴性T细胞和B细胞群体的特征，对自身免疫淋巴增殖性综合征(ALPS)患者及其大家庭成员继续进行广泛的流式细胞术评估。双阴性T细胞被证实为α-βTCR、CD57+、HLA-DR+和CD45RA+。这项研究已经扩展到更完整地描述所有阿尔卑斯病患者的双阴性T细胞，包括B220表达和γ-TCRT细胞。此外，我们已经开始了对B细胞的扩展表征，针对这些患者的记忆B细胞使用CD27和B220评估。最近，根据最新的信息，我们发现了一种相对缺乏的CD4/CD25 T细胞，这可能与这种疾病的自身免疫有关，这种T细胞是一种关键的免疫调节T细胞。针对这一T细胞亚群的功能研究将在不久的将来启动。

An extensive flow cytometric evaluation continues of patients with autoimmune lymphoproliferative syndrome (ALPS) and their extended family members, on the basis of characterization of the expanded double-negative T-cell and B-cell populations. Double-negative T-cells have been demonstrated to be alpha beta TcR, CD57+, HLA-DR+, and CD45RA+. This study has been extended to characterize the double-negative T-cells more completely including B220 expression and gamma-delta TcR T-cells in all ALPS patients. In addition, we have initiated expanded characterization of the B cells, directed at memory B cells using CD27 and B220 assessment in these patients. More recently, we have identified a relative deficiency in CD4/CD25 T cells that could be associated with the autoimmunity in this disorder based on recent information identifying this T cell as a critical immunoregulatory T cell. Functional studies directed at this T cell subpopulation will be initiated in the near future.