Comparison of sickle cell-beta§ thalassaemia with homozygous sickle cell disease

ABSTRACT

Clinical and haematological features in 41 patients with sickle cell-᧠thalassaemia (S᧠thalassaemia) and in 123 age-sex matched controls with homozygous sickle cell (SS) disease were compared. Persistence of splenomegaly was more common and fetal loss less common in S᧠thalassaemia but other clinical features were similar in the two genotypes. Total haemoglobin, Hb A2, PCV, CCV, and red cell count were significantly higher and MCV, MCH, MCHC,and ISC counts significantly lower in Sá§thalassaemia. Proportional reticulocyte counts were significantly lower in S᧠thalassaemia but there was a difference in absolute reticulocyte counts. Persistence of splenomegaly and low ISC counts are compatible with decreased intravascular sickling which may result from the lower mean cell haemoglobin S concentration in S᧠thalassaemia. If beneficial effects of a low MCHC can be confirmed then a carefully monitored trial of iron deficiency in SS disease may be a logical experimental procedure.(AU)