Sickle-cell Anaemia,Sickle-cell Thalassaemia,Sickle-cell Haemoglobin C Disease,and Asymptomatic Haemoglobin C Thalassaemia in one Ghanaian Family

A Ghanaian family is described in which a sickle-cell haemoglobin C man married to a sickle-cell thalassaemia woman produced 12 children (eight alive). Four children have sickle-cell anaemia, two sickle-cell haemoglobin C disease, one has sickle-cell thalassaemia, and one is asymptomatic haemoglobin C thalassaemia.It is emphasized that the contribution that adult sickle-cell disease patients make, through procreation, to the persistence of the S gene may be greater than is normally supposed, and that this contribution may soon outstrip that made by balanced polymorphism through falciparum malaria. Widespread haemoglobin genotyping in schools leading to genetic counselling is advocated to decrease the incidence of sickle-cell disease.     

Acid–Base Status of Adults with Sickle-cell Anaemia

Determinations of the acid–base status of 10 adult Jamaican patients with sickle-cell anaemia during “painful crisis” and after recovery showed no evidence of metabolic acidosis in the former, in contrast to reports from elsewhere. These results could explain the failure of alkalis to abort or alter the acute painful episodes of most patients with sickle-cell anaemia.     

Intraerythrocytic Precipitations of Haemoglobins S and C

HEINZ bodies—intraerythrocytic aggregations of denatured haemoglobin—are characteristically associated with the unstable haemoglobins (Hb), but not with other variants, such as Hbs S or C. In electron microscopy¹, the initial Heinz body lesion consists of small precipitations of relatively unaltered haemoglobin. As these enlarge, coalesce and are fixed to erythrocyte membrane, they can be demonstrated by vital staining; eventually they lead to fragmentation of the cell. Precipitations of the unstable Hb Sabine—even those not visible in vital staining—are easily seen in dark field microscopy of unfixed erythrocytes, especially with illumination from a mercury burner². We now report on such precipitations of Hbs S or C.     

Benign Obstetric History in Women with Sickle-cell Anaemia Associated with α-Thalassaemia

Two Ghanaian women with sickle-cell anaemia and α-thalassaemia were found to have an unusually benign obstetric history. In addition to two factors present which are known to moderate the clinical course of sickle-cell anaemia, good socioeconomic status and a relatively high Hb F level, it is suggested that α-thalassaemia may act among other things by lowering the haemoglobin concentration in the red cells and thereby lowering their tendency to sickle in vivo.