Assessment of Hydrogen Peroxide Generation in Neutrophils

先前的研究证实，基于二氢何达明（DHR）的氧化爆发流式细胞测定是一种评估可能患有慢性肉芽肿病（CGD）患者吞噬细胞的灵敏方法。它还可以作为筛选可能的x连锁CGD携带者的准确测试，并作为疾病基因型的初步筛选。该方法经过优化，对1万个或更少的异常细胞中的一个正常细胞敏感。该项目先前证明了在输注同种异体白细胞后追踪CGD患者正常粒细胞的能力，该试验已被证明在确定输注细胞存活时间和全致敏性方面是可靠的。目前阶段的CGD基因治疗继续针对x连锁CGD患者。DHR测定是一种关键的方法，用于确定循环中基因校正的粒细胞的存在，并用于监测治疗患者基因校正细胞产生的持续时间。

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 as a preliminary screen for the genotype of the disease. The method has been optimized and is sensitive to one normal cell in 10,000 or fewer abnormal cells. This project previously demonstrated the capacity to follow normal granulocytes after allogenic leukocyte transfusion in patients with CGD, and this assay has proven to be reliable in establishing duration of transfused cell survival and all-sensitization. The current phase of gene therapy of CGD continues to be 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 for monitoring the duration of production of the gene-corrected cells in the treated patients.