血红蛋白Constant Spring及CS型血红蛋白H病

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血红蛋白Constant Spring (HbCS)是一种α链异常的血红蛋白。因单纯携带此种异常Hb的患者,临床症状常不明显,故不易被发现,但与其他异常Hb病(如HbH病)并存,症状加重时便较易发现。最早是1971年Paul在牙买加的Constant Spring地区内一华侨家中发现的,因此命名为HbCS,该家系中的患者合并有HbH病。同年,Clegg等对HbCS进行了结构分析,确认其为α链C末端延长的异常Hb。在这之前,于希腊、泰国、马来亚以及香港等地曾先后报导了HbH病患者的红细饱中存在一种特殊慢泳成分,当时曾分别称为Hb Athens、Hb Thai、HbX以及慢泳Hb等,后经结构分析,均证实为HbCS。由 Hemoglobin Constant Spring (HbCS) is an a-chain abnormal hemoglobin. Due to simple Hb patients with such abnormalities, clinical symptoms are often not obvious, it is not easy to be found, but with other abnormal Hb disease (such as HbH disease) coexist, the symptoms are more likely to be found. First discovered in 1971 by Paul, an overseas Chinese in the Constant Spring area of ​​Jamaica, he was named HbCS, and patients in the pedigree had HbH disease. In the same year, Clegg et al. Conducted a structural analysis of HbCS to confirm that it is an abnormal Hb prolonged C-terminal extension of the α chain. Prior to this, there were reports in Greece, Thailand, Malaya and Hong Kong that there was a special slow-swimming component in the redness of HbH patients, then known as Hb Athens, Hb Thai, HbX, and then slowly Swimming Hb, etc., after structural analysis, were confirmed as HbCS. by
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