Mild Clinical Course in Two South African Cases of Sickle-Cell Anaemia

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Abstract
Clinical, haematological and gene mapping data are presented on two South African cases of sickle-cell anaemia. Both individuals, who are siblings, have experienced a very mild clinical course. Restriction endonuclease analysis showed that 1 sibling was homozygous for α+-thalassaemia (genotype α-/α-), whereas the other had a full complement of α-globin genes. Both showed markedly elevated levels of Hb F. The maintenance of high levels of Hb F is the most probable explanation for the moderate clinical expression of the disorder in these patients.