Sickle cell anaemia in Sudan: clinical findings, haematological and serum variables

Summary

Ninety homozygous patients with Hb S haemoglobinopathy from Sudan were reported with respect to clinical findings, haematological and serum parameters. For comparison, 27 Hb AS heterozygous subjects and 28 Hb AA controls were investigated also. The patients showed an extreme type of illness presenting with severe clinical signs such as dactylitis, liver enlargement and cardiac complications. There was marked haemolytic anaemia (mean haemoglobin 66 g/1). Some patients also presented with low serum (S)-iron levels, indicating iron deficiency. S-bilirubin, S-ASAT, S-ALAT, S-GT and S-urate were notably raised, most probably as a consequence of haemolysis with liver involvement. The patients had lower S-calcium levels when compared with the AS and AA subjects. This may suggest a possible role of HB S in the production of this feature.