Abstract
Sickle beta-thalassaemia (S beta-thalassaemia) is a condition, which results from coinheritance of a sickle cell gene and a beta-thalassaemia gene. The clinical phenotype depends on the type of beta-thalassaemia gene (beta+ or beta(o)). There are several similarities in clinical and haematological features, which sometimes pose a difficulty in correct diagnosis. A definitive diagnosis is required in order to initiate early supportive treatment in patients with homozygous sickle cell disease (SS disease) and to define the later clinical course. Forty-seven cases of haemoglobin sickle syndrome (HbS syndrome) were studied. The clinico-haematological features and high-performance liquid chromatography (HPLC) results from 17 patients with S beta-thalassaemia were compared with those of SS disease (10 patients). Splenomegaly was more commonly found in patients with S beta-thalassaemia. Among the haematological features, red blood cell counts and HbA2 levels were significantly higher in patients with S beta-thalassaemia, while red cell indices, such as MCV, MCH were significantly lower than those seen SS disease. MCHC, PCV total haemoglobin (Hb), HbS, A and HbF were similar in the two groups.
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