Thalassaemia Major with Complete Suppression of HbA Production in a Ghanaian Girl

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Abstract
A 7-year-old Ghanaian girl showed severe hypochromic anaemia, hepatosplenomegaly, widening of the diploic space and a hair on end appearance on a skull X-ray. Hb electrophoresis revealed only one thick band, which was HbF, no HbA was present. Both the mother and her brother are β-thalassaemia traits; it was impossible to investigate the father. Very few communications are available on thalassaemia major among Negroes. The view is expressed that there is a heterogeneity of the thalassaemia gene in Africans, and all the cases published previously have HbA in addition to HbF. The present case belong to another group where the β -chain is suppressed completely. It is assumed that in Africa, children with thalassaemia major die in early childhood without proper diagnosis and only those survive who have a milder course, however, those may also die before they reach the end of the first decade. Further case reports and clinical and laboratory studies are necessary to obtain more information on thalassaemia major in Africans.

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