Red cell deformability, splenic function and anaemia in thalassaemia

Abstract

Red cell deformability (RCD) was measured in 38 patients with α- thalassaemia and 48 patients with β-thalassaemia, of whom 13 had undergone splenectomy. All splenectomized patients, but none of those with intact spleens, had very rigid erythrocytes with an elongation index <0.45 at a high shear stress of 30 Pa suggesting a splenic recognition threshold for removal of rigid red cells. At this shear stress RCD correlated strongly with the degree of anaemia in both the splenectomized (r = 0.81, P < 0.001) and non- splenectomized β-thalassaemic patients (all patients r=0.81, P<0.001; homozygous β-thalassaemic patients r= 0.51, P= 0.01). These data suggest that reduced RCD is a major determinant of anaemia in thalassaemia.