Assessment Of Lymphocytes In Patients With Autoimmune Ly

在扩增双阴性t细胞和b细胞群特征的基础上，对自身免疫性淋巴细胞增生性综合征（ALPS）患者及其大家庭成员进行了广泛的流式细胞术评估。双阴性t细胞已被证明是α - β TcR、CD57+、HLA-DR+和CD45RA+。这项研究已经扩展到更完整地表征双阴性t细胞，包括B220表达和γ - δ TcR t细胞在所有ALPS患者中的表达。此外，我们已经开始扩大B细胞的表征，针对这些患者使用CD27和B220评估记忆B细胞。在ALPS患者的B细胞中观察到的结果与另一项针对人淋巴细胞B220表达评估的活性方案直接相关。我们已经确定的CD4/CD25 T细胞的相对缺乏导致了针对该T细胞亚群的功能研究的开始，以评估免疫表型发现是否代表免疫调节性T细胞的功能缺陷，从而可以解释ALPS 1a型家庭的基因型表型差异。

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.