Coots M C, Glueck H I
Department of Pathology and Laboratory Medicine, University of Cincinnati College of Medicine, OH 45267-0529.
Am J Hematol. 1990 Oct;35(2):73-9. doi: 10.1002/ajh.2830350202.
The purpose of this study was to determine if factor VIII:C inhibitors completely disappear during remission and if they recur on exacerbation, are they similar to or different from the original inhibitors? To answer these questions, isofocusing, which separates proteins on the basis of their pIs, was utilized to recover inhibitors from the plasmas of six patients, five nonhemophiliacs with acquired VIII:C inhibitors and the sixth with classic hemophilia. Initially, all plasmas were studied during the presenting hemorrhagic episodes and, subsequently, following the disappearance or recurrence of the inhibitors. Following isofocusing, each fraction was tested for inhibitory activity. The method enabled us to determine that inhibitors to factor VIII:C persisted even when they could not be demonstrated by conventional methods. The inhibitory activity resulted from a composite of inhibitors, each of the group identified by its own pI. In many patients, some peaks persisted throughout the entire interval of study, whereas others disappeared and new ones appeared, suggesting that various groups of cells were capable of producing the inhibitors.
本研究的目的是确定凝血因子VIII：C抑制剂在缓解期是否完全消失，以及在病情加重时是否复发，复发的抑制剂与原始抑制剂是否相似或不同？为了回答这些问题，利用等电聚焦技术（根据蛋白质的等电点分离蛋白质）从六名患者的血浆中回收抑制剂，其中五名非血友病患者患有获得性VIII：C抑制剂，第六名患有经典血友病。最初，在出现出血发作期间对所有血浆进行研究，随后，在抑制剂消失或复发后进行研究。等电聚焦后，对每个馏分进行抑制活性测试。该方法使我们能够确定，即使凝血因子VIII：C抑制剂无法通过传统方法检测到，它们仍然存在。抑制活性是由多种抑制剂组成的，每组抑制剂都由其自身的等电点确定。在许多患者中，一些峰在整个研究期间持续存在，而其他峰消失并出现新的峰，这表明不同组的细胞能够产生抑制剂。
