Subclassification of HbS syndrome: is it necessary?
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.
Tyagi
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S
Department of Haematology, All India Institute of Medical Sciences, New Delhi, India. drseematyagi@hotmail.com
Choudhry
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VP
Saxena
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R
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000145:classification* / Q000453:epidemiology / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D004906:Erythrocyte Count; D004909:Erythrocyte Indices; D005260:Female; D005914:Globins / Q000235:genetics; D006454:Hemoglobins / Q000032:analysis; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007194:India / Q000453:epidemiology; D007565:Jaundice / Q000209:etiology; D007871:Leg Ulcer / Q000209:etiology; D008297:Male; D010146:Pain / Q000209:etiology; D012805:Sickle Cell Trait / Q000453:epidemiology / Q000235:genetics; D013163:Splenomegaly / Q000209:etiology; D013577:Syndrome; D019851:Thrombophilia / Q000209:etiology; D017086:beta-Thalassemia / Q000097:blood / Q000145:classification / Q000453:epidemiology / Q000235:genetics*
D006454:Hemoglobins; D005914:Globins