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本文应用限制性内切酶、α珠蛋白基因探针分析了24例HbH病,并进行了临床观察和血液学分析;结果9例为非缺失型与α地贫_1双重杂合子,5例为HbCS与α地贫_1双重杂合子;他们病情重,有11例作了牌切除。另10例是α地贫_2与α地贫_1双重杂合子,临床症状和贫血程度轻,脾切除的仅1例。
In this paper, 24 cases of HbH disease were analyzed by using restriction endonuclease and α-globin gene probe, and clinical observation and hematological analysis were performed. Results Nine cases were non-deletion type and α-thalassemia-1 double heterozygote, and 5 cases HbCS and α thalassemia _1 double heterozygotes; they are seriously ill, 11 cases were card resection. The other 10 cases were α-thalassemia-2 and α-thalassemia-1 double heterozygote. Clinical symptoms and anemia were mild and only 1 case was splenectomy.