Assessment of Hydrogen Peroxide Generation in Neutrophils

以往的研究证实，基于双氢罗丹明(DHR)的流式细胞仪检测氧化爆发是评估慢性肉芽肿病(CGD)患者吞噬细胞的一种敏感方法。它也被用作筛查可能的X连锁CGD携带者的准确测试，以及作为疾病基因的初步筛查。对该方法进行了优化，对10,000个或更少的异常细胞中有一个正常细胞进行了检测。该项目先前证明了在CGD患者输注同种异体白细胞后跟随正常粒细胞的能力，该检测方法已被证明在确定输注细胞存活和ALL致敏的持续时间方面是可靠的。目前CGD的基因治疗阶段继续针对X连锁CGD患者。DHR试验正被用作确定循环中是否存在基因校正的粒细胞以及监测接受治疗的患者中基因校正的细胞产生时间的关键方法。

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 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.