Vnitr Lek 1992, 38(6):598-602

[New views on the etiopathogenesis of beta-thalassemia].

V Fábryová: Oddelenie hematológie a transfúziológie, NsP Staré Mesto, Bratislava.

In the submitted paper the author deals with recent findings on damage of erythrocyte membranes and enzymes in beta-thalassemia. The primary disorder in the formation of beta chains leads to the formation of haemichromes and free oxygen radicals which leads to peroxidative damage of the lipid membrane. The thus generated aldehydes damage important thiol groups of enzymes. Haemichromes interfere with the structure of the protein membrane and the cytoskeleton of the cell. There are differences in membrane damage between alpha and beta thalassaemia. Beta-thalassaemia must be conceived as a comprehensive disease of red blood cells not only as a haemoglobinopathy.