125例地中海贫血杂合子临床血液学分析

Clinical Hematological Analysis of 125 Heterozygotes for Thalassemias

目的对１２５例不同类型的地中海贫血（地贫）杂合子进行临床血液学分析。方法用Ｃｏｕｌｔｅｒ血球计数仪测定分析对象末梢血红细胞平均容量（ＭＣＶ）和红细胞平均血红蛋白（ＭＣＨ）。用醋纤薄膜电泳比色法及１分钟碱变性法分别测得ＨｂＡ２和ＨｂＦ含量。结果所有地贫杂合子的ＭＣＶ、ＭＣＨ明显小于正常值（Ｐ＜０．００１），符合小细胞低色素性贫血，同时发现不同的β地贫突变类型，其ＭＣＶ也存在差异，β０突变杂合子ＭＣＶ低于β＋突变杂合子（Ｐ＜０．０３）。结论ＭＣＶ和ＭＣＨ的测定不仅可为地贫的临床诊断提供依据。

Objective To analyze the clinical hematological data of 125 heterozygotes for different kinds of thalassemias.Method Mean corpuscular volume (MCV) and Mean corpusular hemoglobin (MCH) of 125 heterozygotes for thalassemias were measured by an automatic Coulter analyzer.Hemoglobin A2 was determined spectrophotometrically after electrophoretic separation on cellulose acetate membrane.Hemoglobin F was determined by alkali denaturation test.Results Mean MCV and MCH in all the tested thalassemia heterozygotes were statistically significantly lower than those in normals (P<0.001).The result also showed that there was a significant difference of the MCV value between different βthalassemia mutations,i.e.,Mean MCV of β0thalassemia heterozygotes was statistically significantly lower than β+thalassemia heterozygotes (P<0.03).Conclusions The MCV and MCH are valuable parameters in clinical diagnosis for thalassemia heterozygotes.In addition,MCV provides information for the identification of thalassemia heterozygotes with mutational diversity.