珠蛋白生成障碍性贫血患者红细胞游离原卟啉水平的研究

测定116例珠蛋白生成障碍性贫血、37例缺铁性贫血患者及45例正常人的FEP水平。结果:6例(9.2％)杂合子β珠蛋白生成障碍性贫血病人的FEP增高,其中4例合并缺铁;14例(27.5％)纯合子β珠蛋白生成障碍性贫血、HbH病及β珠蛋白生成障碍性贫血复合HbE病患者的FEP增高,与缺铁无关。缺铁性贫血病人的FEP水平显著高于杂合子β珠蛋白生成障碍性贫血病人。37例缺铁性贫血病人中,34例(91.9％)FEP增高;而37例非缺铁的杂合子β珠蛋白生成障碍性贫血患者中,36例(97.3％)FEP在正常范围,FEP测定对两者的鉴别诊断有一定的临床价值。

A STUDY OF FREE ERYTHROCYTE PROTOPORPHYRIN IN THALASSEMIA

Free erythrocyte Protoporphyrin(FEP)were determined in 116 thalassemia patients,37 iron deficiency anemia patients and 45 normal subjects.The FEP values were increased in 6(9.2%)beta heterozygous thalassemia patients.Among these patients,four were related to iron depletion.The FEP values were increased in 14(27.5%)cases with beta homozygous thalassemia,hemoglobin H disease and HbE-beta thalassemia patients.None of these patients was related to iron depletion.The mean FEP values in the iron deficiency anemia group were significantly higher than those of the beta heterozygous thalassemia group.The FEP values were increased in 34(91.9%)patients with iron deficiency anemia,but were within the normal range in 36(97.3%)none iron depletion beta heterozygous thalassemia patients.Measurement of FEP is a simple means for distinguishing iron deficiency anemia from beta heterozygous thalassemia.