THE INFLUENCE OF ALPHA-THALASSEMIA ON THE HEMATOLOGICAL AND CLINICAL EXPRESSION OF SICKLE-CELL DISEASE IN WESTERN INDIA

We evaluated the clinical and haematological features of 29 sickle cel l anaemia patients with associated alpha-thalassaemia and 22 sickle ce ll homozygotes with a normal alpha-globin genotype from western India. The presence of alpha-thalassaemia resulted in significantly higher h aemoglobin (Hb), haematocrit (HCT), red blood cells counts (RBC) and h aemoglobin A(2) (HbA(2)) levels but lower mean cell haemoglobin (MCH) and mean cell volume (MCV). The clinical presentation in these patient s was also milder with fewer episodes of painful crisis, chest syndrom es, infections, requirement of hospitalization and blood transfusions. However, splenomegaly was more common as compared to the patients wit h a normal alpha-globin genotype. It is evident from the present study that alpha-thalassaemia could be an important genetic factor modulati ng the clinical expression and haematological severity of sickle cell anaemia in this region.