The incidence and clinical effects of abnormal haemoglobin in Jamaica (abstract only)

ABSTRACT

The mode of inheritance of the genes carrying abnormal haemoglobins is discussed, together with the methods used for their study. Some of our findings in Jamaica during the last nine months are presented. 780 women attending the ante-natal clinic have been investigated by haemoglobin electrophoresis. The incidence of the sickle cell trait is 9.5 per cent and of the haemoglobin C trait 3.4 per cent. In addition two women were found to have unsuspected sickle cell/haemoglobin C disease, and one lady had apparent sickle cell anaemia - she has four healthy children. The clinical features of sickle cell anaemia and other related diseases are discussed. Three cases of Cooley's Anaemia are reported, but the incidence of the Cooley gene is not known. The importance of this gene is stressed, for in conjunction with the S gene a condition - sickle cell/thalassaemia disease - is produced which may be indistinguishable clinically and in the laboratory from sickle cell anaemia. A case of sickle cell/thalassaemia disease is reported in which normal haemoglobin (A) production is completely suppressed giving the same electrophoretic and haematological picture as is found in sickle cell anaemia. The importance of differentiating this condition from sickle cell anaemia is stressed. This can be done by family studies. An example of homozygous haemoglobin C disease is reported. (AU)