[Clinical and biological aspects of beta-thalassemia. Apropos of 176 cases].
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.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D006441:Hemoglobin A / Q000032:analysis; D006444:Hemoglobin C / Q000032:analysis; D006445:Hemoglobin C Disease / Q000097:blood / Q000150:complications / Q000235:genetics; D006451:Hemoglobin, Sickle / Q000032:analysis; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007223:Infant; D008297:Male; D013789:Thalassemia / Q000097:blood / Q000150:complications / Q000235:genetics
D006451:Hemoglobin, Sickle; D006444:Hemoglobin C; D006441:Hemoglobin A
Aspects cliniques et biologiques des bêta-thalassémies. A propos de 176 observations.