The pathology of sickle cell haemoglobin C disease and sickle cell anaemia.

The purpose of this communication is to describe the necropsy findings in three patients dying of sickle cell haemoglobin C disease and two of sickle cell anaemia. In southern Ghana there is a high incidence of abnormal haemoglobins in the population, the incidence of haemoglobin S being 18% and of haemoglobin C 12% (Edington and Lehmann, 1954a). Haemoglobin G has also been described (Edington and Lehmann, 1954b), a form of thalassaemia is present (Edington and Lehmann, 1955), and congenital haemolytic icterus has been diagnosed. Little information is available on the pathology of the " mixed abnormal haemoglobin diseases " and, indeed, the discovery of haemoglobin C by Itano and Neel (1950) invalidates much of the pathology attributed to sickle cell anaemia before this time, as sickle cell anaemia was not distinguished from sickle cell haemoglobin C disease. In a previous communication (Edington, 1955) the pathology of sickle cell disease in West Africa was discussed. This term was employed to describe those conditions in which the sickling phenomenon was considered to be a factor in the cause of death. At necropsy two different types of lesion were demonstrable in the spleen. In some cases death oCcurred in subjects in which the small siderofibrotic type of spleen was found and the cause of death in these instances was considered to be sickle cell anaemia. In others the spleen was grossly enlarged and congested and death was believed to be due to a "sickle cell crisis." It was pointed out that the sickle cell crisis in many instances was not likely to be due to the homozygous inheritance of the S gene, but probably represented a number of conditions in which the sickle cell trait was in combination with other inherited anomalies of the blood. In an attempt to elucidate the pathology of some of the abnormal haemoglobin diseases paper electrophoresis was performed on the blood of 99 subjects coming to necropsy and the following