Objective: To analyze the hematological characteristics of Chinese Gγ+(Aγδβ)0-thalassemia，SEA-HPFH and Taiwan type β-thalassemia.
Methods: Hemoglobin electrophoresis and blood routine test were used to analyze the hematological indexes of all peripheral blood samples，PCR-Flow fluorescent hybridization and Gap-PCR were used to detect the globin gene mutations and the data were analyzed statistically.
Results: The 3 types of deletion β- Thalassemia patients were showed as hypochromic small cell anemia. The MCH and MCV values of Taiwan type β-thalassemia patients were the lowest. The results of hemoglobin electrophoresis showed that the increasing of HbF was found in all of the 3 types. Except for the decreasing of Hb A2 in Chinese Gγ+(Aγδβ)0-thalassemia，the levels of Hb A2 in the other two deletion β-thalassemia patients were significantly increased. Except for Hb, there were significant differences in MCV, MCH, Hb A2 and HbF between Chinese Gγ+(Aγδβ)0-thalassemia and SEA-HPFH(P<0.001).
Conclusion: Through analyze the hematological characteristics, it can be provide that the guidance for the differential diagnosis and genetic consultation of the three commonest deletion β-thalassemia in Chinese.
题目: 中国人3种最常见缺失型β地中海贫血的鉴别诊断.
目的: 分析中国型Gγ+(Aγδβ)0 地中海贫血、东南亚型遗传性胎儿血红蛋白持续增高症（SEA-HPFH）和台湾型β地中海贫血患者的血液学特征.
方法: 应用血红蛋白电泳和血常规检测对所有采集的外周血标本进行血液学指标分析，使用PCR-流式荧光杂交法和Gap-PCR的方法进行珠蛋白基因突变检测，并对相关血液学参数进行统计学分析.
结果: 3种缺失型β地中海贫血患者均表现为小细胞低色素性贫血，其中台湾型β地中海贫血患者的MCH和MCV值最低。血红蛋白电泳结果显示，3者都有显著增高的HbF，除中国型Gγ+(Aγδβ)0 地中海贫血患者的HbA2值有所降低外，其他2种缺失型β地贫患者的HbA2水平都有明显升高。中国型Gγ+(Aγδβ)0 地中海贫血杂合子和SEA-HPFH杂合子的患者除Hb无统计学差异外（P=0.591），MCV、MCH、HbA2及HbF值均有统计学差异（P<0.001）.
结论: 通过对中国人3种最常见缺失型β地中海贫血患者血液学特性的分析，可以为其鉴别诊断和遗传咨询提供指导.