广西地区104例泰国缺失型alpha-地中海贫血1 的研究

目的：对广西地区的泰国缺失型琢-地中海贫血1 的血液学、基因型、临床表现及民族分布进行分析。方法：对门诊病人进行 血常规、血红蛋白电泳及- 地中海贫血基因分析，收集已确诊为泰国缺失型琢- 地中海贫血1 患者的检测数据及临床资料，并用 SPSS 统计软件对以上数据进行统计分析。结果：共检出104 例泰国缺失型alpha- 地中海贫血1，其中71 例基因型为--THAI /alpha-alpha，17 例 基因型为--THAI/-alpha3.7，14 例基因型为--THAI/alpha CS alpha ，2 例基因型为--THAI/alpha QS alpha。统计学分析：--THAI/alpha alpha杂合子和--SEA/alpha-alpha杂合子的血常规 结果比较没有统计学意义，P>0.05；泰国型Hb H病(--THAI/-alpha3.7、--THAI/alpha-CSalpha、--THAI/alpha-CSalpha)和东南亚型Hb H病（--SEA/-alpha-alpha3.7、--SEA/alpha-CS-alpha)的血常 规结果比较有统计学意义，P<0.01；民族分布上，有67 例为壮族，34 例为汉族，其他民族3 例。结论：泰国缺失型- 地中海贫血1 在 广西有一定的发生率，在壮族人群多见；和东南亚缺失型Hb H病相比，泰国缺失型Hb H病出现临床症状的时间更早、贫血更严 重。

Analysis of 104 Cases of Thai Type Deletionalpha-thalassemia1in Guangxi Region

Objective:To analyze hematology, genotype, phenotype and ethnic distribution of Thai type deletions thalassemia in Guangxi region.Methods:The outpatient''s blood, hemoglobin and -thalassemia genotype was detected and these data were collected and analyzed.Results:104 cases of Thai type deletions thalassemia were detected, including 71 cases of --Thai/alpha-alpha, 17 cases of -THAI/-alpha3.7, 14 cases of --THAI/alphaCSalpha, 2 cases of --THAI/alphaQSalpha; statistically significant difference were not fund between --THAI/alpha-alpha and --SEA/alpha-alpha in blood test, P>0.05, statistically significant difference were found between Thai type deletions Hb H disease (--THAI/-alpha3.7,--THAI/alphaCSalpha, --THAI/alphaQSalpha) and Southeast Asia deletions type Hb H disease (--SEA/-alpha3.7, --SEA/alphaCSalpha), P<0.01. The ethnic distribution were found in 67 cases of Zhuang, 34 cases of Han, three cases of other ethnics.Conclusion:Thai type deletions thalassemia has a certain incidence in Guangxi, most of Zhuang people. Compared to Southeast deletions Hb H disease, Thai type deletions Hb H disease (--Thai/alphaCSalpha) showed an earlier, severe symptoms of anemia.