Effect of alpha thalassaemia on the rheology of homozygous sickle cell disease

A study of rheological determinants (plasma viscosity, whole-blood viscosity, and erythrocyte deformability) was made in 24 matched pairs of patients with homozygous sickle cell disease, with and without homozygous x-thalassaemia. Patients with coexisting x-thalassaemia showed a significant increase in erythrocyte deformability measured as filtration of washed erythrocytes through 5 um diameter pores and also as viscosity of whole blood at high shear rate (230s-1) and standard haematocrit (0.45). This rheological advantage may explain the beneficial effect of x-thalassaemia 2 on haematological parameters and clinical events in homozygous sickle cell disease (AU)