Sickle cell beta-thalassaemia compared with sickle cell anaemia in Algeria

Clinical, haematological and biochemical features in 42 subjects with S-beta thalassaemia (31 subjects with S-beta thalassaemia and 11 subjects with S-beta+ thalassaemia); and in 42 with homozygous sickle cell disease were compared. Persistent splenomegaly was more common and painful crises less com...

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Published inScandinavian journal of haematology Vol. 32; no. 4; p. 346
Main Authors Belhani, M, Morle, L, Godet, J, Bachir, D, Henni, T, Zerhouni, F, Bensenouci, A, Colonna, P
Format Journal Article
LanguageEnglish
Published Denmark 01.04.1984
Subjects
Adolescent
Algeria
Anemia, Sickle Cell - genetics
Anemia, Sickle Cell - pathology
Blood Transfusion
Female
Globins - biosynthesis
Hemoglobin A - analysis
Heterozygote
Humans
Male
Sex Ratio
Splenectomy
Splenomegaly - etiology
Thalassemia - genetics
Thalassemia - pathology
Thalassemia - therapy
Algeria
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Summary:Clinical, haematological and biochemical features in 42 subjects with S-beta thalassaemia (31 subjects with S-beta thalassaemia and 11 subjects with S-beta+ thalassaemia); and in 42 with homozygous sickle cell disease were compared. Persistent splenomegaly was more common and painful crises less common in the S-beta thalassaemia group. Total Hb was higher and reticulocyte count lower in S-beta+ thalassaemia than in S-beta thalassaemia or SS disease. Microcytosis was marked in the S-beta thalassaemia group while the MCV was normal in sickle cell anaemia. Hb F was significantly higher in the S-beta thalassaemia group, without any influence on the severity of the disease. Many features suggest that sickle cell thalassaemia is more severe in Algeria than in Negro subjects and similar to the disease in Italian patients.
ISSN:0036-553X
DOI:10.1111/j.1600-0609.1984.tb00687.x