Tejuca M, Martinez G, Ferreira R, Hernández A, Ferrer L, Goicochea A, Hernández A C, Coyula R, Wade M, Colombo B
Institute of Hematology and Immunology, Habana, Cuba.
Haematologia (Budap). 1989;22(3):175-80.
Several factors are implied in the haematological and clinical picture of sickle cell anaemia. Attention has been focused on the concomitant presence of -alpha-thalassemia and high levels of HbF, but contradictory results have been reported in different populations. We compared the blood cell density profile, obtained by the phtalate esther method, of normal subjects with those of patients with sickle cell anaemia - with or without heterozygous alpha-thalassemia. We found that the density profile of both groups of patients differs from normal subjects, and that a difference can also be demonstrated between normal alpha genotype patients with sickle cell anaemia and patients with heterozygous alpha-thalassemia. These results are in agreement with the findings obtained in other countries in which a gene from Caucasian to African populations have been demonstrated, and are different from the results obtained in populations of more pure African ancestry. It can be suggested, therefore, that these data, in addition with findings of other authors in different geographical areas, support the hypothesis that the genetic make up plays an important role in the haematologic and clinical picture of sickle cell anaemia.
镰状细胞贫血的血液学和临床症状涉及多个因素。人们一直关注伴发的α地中海贫血和高水平的胎儿血红蛋白(HbF),但不同人群的研究结果相互矛盾。我们采用邻苯二甲酸酯法比较了正常受试者与患有或未患有杂合子α地中海贫血的镰状细胞贫血患者的血细胞密度分布。我们发现,两组患者的密度分布均与正常受试者不同,并且患有镰状细胞贫血的正常α基因型患者与杂合子α地中海贫血患者之间也存在差异。这些结果与其他国家的研究结果一致,在这些国家已证明存在从白种人群到非洲人群的基因转移,并且与非洲血统更纯正的人群的研究结果不同。因此,可以认为,这些数据以及其他作者在不同地理区域的研究结果支持了以下假设:基因组成在镰状细胞贫血的血液学和临床症状中起着重要作用。