The pathology of sickle-cell disease in West Africa

Abstract

The autopsy records in the Medical Research Institute, Accra have been examined. In 2,880 autopsies the sickling phenomenon was considered to be a factor in the cause of death of 104 subjects (3.6 per cent.).The incidence of the trait in 293 necropsies was 21 per cent.Blocks of tissues were available in 40 autopsies and the pathology seen in these autopsies has been described. Classical sickle-cell anaemia occurred, but in the majority of the autopsies the spleen was enlarged and congested.The pathological lesions seen were of two types.In the first instance signs of haemolysis might be marked and were most severe in those subjects in which the small siderofibrotic spleen of sickle-cell anaemia was seen, or they might be minimal or absent even in the presence of erythrophagocytosis. Secondly blockage of the smaller vessels by sickled erythrocytes occurred and was responsible for a wide range of pathological lesions. Thirteen pregnant women died suddenly in the last three months of pregnancy : at autopsy the spleen was enlarged and congested, the remaining organs, with the exception of the liver, exhibiting gross pallor. Five babies born of these mothers did not exhibit the sickle-cell trait, proving that the mothers were not homozygous sicklers. In addition electro-phoretic analysis of the blood in one of these women revealed the presence of the trait only ; one other pregnant woman, suffering from sickle-cell haemoglobin C disease, died suddenly in crisis but no necropsy was obtained. The occurrence of the sickle-cell crisis in individuals heterozygous for the trait will most probably be explained by the presence of an additional abnormal haemoglobin or, less likely, by the presence of a relatively high percentage of sickle-cell haemoglobin in the erythron, the crisis being precipitated by conditions or infections inducing anoxaemia. The term sickle-cell disease has been employed to describe all autopsies in which the sickling phenomenon has been implicated in the cause of death and in which a characteristic pathology has been seen.