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回顾研究175名α珠蛋白合成障碍性贫血患者(包括HbH病人130人,α地Ⅰ型24人,α地Ⅱ型21人)的结果表明:HbH病人多项临床和血液学指标异常,血清SI和TS增高。α地Ⅰ型患者临床仅表现贫血貌;血液学指数MCH、DF值和MCV/RBC比值任意一项阳性,结合家系调查和排除试验可作为α地Ⅰ型筛选指标。本文还发现20%α地Ⅰ型病人H包涵体阳性且H包涵体只出现在α地贫病人,故提高检测灵敏度,H包涵体可能成为α地Ⅰ型简便有效的确诊指标。α地Ⅱ型患者临床和血液学检查未见明显异常。
A retrospective study of 175 patients with alpha-globin synthesis anemia (including 130 patients with HbH, α-type I 24, α-type II 21) showed that: HbH patients with a number of clinical and hematological abnormalities, serum SI And TS increased. α type I patients showed only clinical anemia; hematology index MCH, DF and MCV / RBC ratio of any one positive, combined with pedigree investigation and exclusion tests can be used as α type Ⅰ screening index. This article also found that 20% of patients with α-type H-positive inclusion bodies and H-inclusion bodies only appear in patients with α-thalassemia, so to improve the detection sensitivity, H inclusion body may become α type I simple and effective diagnostic criteria. α type Ⅱ patients with clinical and hematological examination showed no abnormalities.