Lymphocytes In Autoimmune Lymphoproliferative Syndrome

一个广泛的流式细胞术评估继续与自身免疫性淋巴组织增生综合征（ALPS）患者及其大家庭成员，扩展的双阴性T细胞和B细胞群体的特征的基础上。双阴性T细胞已被证明是α β TcR、CD 57+、HLA-DR+和CD 45 RA+。这项研究已经扩展到更完整地表征双阴性T细胞，包括所有ALPS患者中的B220表达和γ-δ TcR T细胞。此外，我们已经启动了B细胞的扩展表征，在这些患者中使用CD 27和B220评估针对记忆B细胞。最近，我们已经确定了CD 4/CD 25 T细胞的相对缺陷，这可能与这种疾病中的自身免疫性相关，基于最近的信息，将这种T细胞鉴定为关键的免疫调节T细胞。针对该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.