Belhani M, Dahmane M, Richard F, Trabuchet G, Benabadji M, Colonna P, Labie D
Sem Hop. 1977 Apr 23;53(16):891-7.
The study of 176 subjects with beta-thalassemia, associated or not with a hemoglobinopathy, shows great diversity. The hemoglobin C thalassemias are less severe and form a fairly homogeneous group. Sickle cell thalassemia cases have more marked anemia and the disease takes on more varied forms, no doubt because the main mechanism of the anemia, the hyperhemolysis, is influenced by several factors which have a variable effect on the clinical picture. Unassociated thalassemias seem the most polymorphic. Although it seems that in certain foci the beta-thalassemias are fairly stereotyped, this first study shows in Algeria great heterogeneity. All forms are observed both clinically and in the laboratory. Present classifications have not supplied a sufficiently operative model. It is not doubt necessary to await further progress in the laboratory to classify these diseases more precisely.
对176名患有β地中海贫血（无论是否伴有血红蛋白病）的受试者进行的研究显示出很大的多样性。血红蛋白C地中海贫血病情较轻，构成一个相当同质的群体。镰状细胞地中海贫血病例有更明显的贫血，且疾病呈现出更多样的形式，这无疑是因为贫血的主要机制——过度溶血——受多种因素影响，这些因素对临床表现有不同作用。未伴有其他疾病的地中海贫血似乎最为多样。尽管在某些地区β地中海贫血似乎相当典型，但这项首次研究表明，在阿尔及利亚存在很大的异质性。所有类型在临床和实验室中均有观察到。目前的分类尚未提供一个足够实用的模型。无疑有必要等待实验室取得进一步进展，以便更精确地对这些疾病进行分类。
