ASSESSMENT OF HYDROGEN PEROXIDE GENERATION IN NEUTROPHILS

先前的工作确定了用于评价氧化爆发的基于流式细胞术的二氢罗丹明（DHR）的测定是用于评价可能患有慢性肉芽肿病（CGD）的患者中的吞噬细胞的敏感方法。它也是有用的，作为一个准确的测试，筛选可能的X-连锁CGD载体和初步筛选的基因型的疾病。该方法经过优化，对小于或等于10，000个异常细胞中的一个正常细胞敏感。开展了一个项目来追踪CGD患者输注异基因白细胞后的输注后粒细胞，该测定法已被证明在确定输注细胞存活和全致敏持续时间方面非常可靠。这项研究的结果在美国血库协会年会上发表。CGD基因治疗的第二阶段继续针对X连锁CGD患者。DHR测定法被用作确定循环中基因校正的粒细胞的存在和监测治疗患者中基因校正的细胞产生的持续时间的关键方法

Previous work established that the flow cytometric dihydrorhodamine (DHR)-based assay for evaluation of oxidative burst is a sensitive method for evaluating phagocytes in patients with possible chronic granulomatous disease (CGD). It has also been useful as an accurate test for screening possible X-linked CGD carriers and a preliminary screen for the genotype of the disease. The method was optimized and is sensitive to one normal cell in less than or equal to 10,000 abnormal cells. A project was undertaken to track granulocytes post-transfusion in CGD patients following allogeneic leukocyte transfusions and this assay has proven to be very reliable in establishing duration of transfused cell survival and allsensitization. The results of this study were presented at the American Association of Blood Banks annual meeting. The second phase of gene therapy of CGD continues directed at patients with X-linked CGD. The DHR assay is being applied as a critical method for establishing the presence of gene-corrected granulocytes in the circulation and monitoring the duration of production of the gene-corrected cells in the treated patients