Table_4_The EuroFlow PID Orientation Tube for Flow Cytometric Diagnostic Screening of Primary Immunodeficiencies of the Lymphoid System.pdf

In the rapidly evolving field of primary immunodeficiencies (PID), the EuroFlow consortium decided to develop a PID orientation and screening tube that facilitates fast, standardized, and validated immunophenotypic diagnosis of lymphoid PID, and allows full exchange of data between centers. Our aim was to develop a tool that would be universal for all lymphoid PIDs and offer high sensitivity to identify a lymphoid PID (without a need for specificity to diagnose particular PID) and to guide and prioritize further diagnostic modalities and clinical management. The tube composition has been defined in a stepwise manner through several cycles of design-testing-evaluation-redesign in a multicenter setting. Equally important appeared to be the standardized pre-analytical procedures (sample preparation and instrument setup), analytical procedures (immunostaining and data acquisition), the software analysis (a multidimensional view based on a reference database in Infinicyt software), and data interpretation. This standardized EuroFlow concept has been tested on 250 healthy controls and 99 PID patients with defined genetic defects. In addition, an application of new EuroFlow software tools with multidimensional pattern recognition was designed with inclusion of maturation pathways in multidimensional patterns (APS plots). The major advantage of the EuroFlow approach is that data can be fully exchanged between different laboratories in any country of the world, which is especially of interest for the PID field, with generally low numbers of cases per center.

在快速发展的原发性免疫缺陷病（PID）领域，EuroFlow联盟决定开发一种PID导向和筛选管，以促进淋巴oid PID的快速、标准化和验证性免疫表型诊断，并允许中心间实现数据的全面交换。我们的目标是开发一种适用于所有淋巴oid PID的通用工具，具有高灵敏度以识别淋巴oid PID（无需对特定PID进行特异性诊断），并指导及优先考虑进一步的诊断手段和临床管理。该管剂的组成通过多中心设置中的设计-测试-评估-再设计几个循环逐步确定。同样重要的是标准化的前分析程序（样本制备和仪器设置）、分析程序（免疫染色和数据采集）、软件分析（基于Infinicyt软件中参考数据库的多维视图）以及数据解读。这一标准化的EuroFlow概念已在250名健康对照者和99名具有明确遗传缺陷的PID患者身上进行了测试。此外，还设计了一种应用新的EuroFlow软件工具的多维模式识别，包括在多维模式中纳入成熟途径（APS图）。EuroFlow方法的主要优势在于，数据可以在世界各地的不同实验室之间完全交换，这对于病例数量普遍较少的PID领域尤其具有吸引力。