Assessment Of Lymphocytes In Patients With Autoimmune Ly

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。在ALPS患者的B细胞中的观察结果与针对评估人淋巴细胞上B220表达的附加活性方案直接相关。我们已经确定的CD 4/CD 25 T细胞的相对缺陷导致了针对该T细胞亚群的功能研究的启动，以评估免疫表型结果是否代表免疫调节T细胞的功能缺陷，这可以解释ALPS 1a型家族的基因型表型差异。

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. The observations in the B cells of ALPS patients are tied directly to an additional active protocol directed at the assessment of B220 expression on human lymphocytes. The relative deficiency in CD4/CD25 T cells that we have identified has resulted in the initiation of functional studies directed at this T cell subpopulation to assess if the immunophenotypic findings represent a functional defect in immunoregulatory T cells that could explain the genotype phenotype disparity in families with ALPS type 1a.