Autoimmune hemolytic anaemia: pattern of presentation and management outcome in a Nigerian population: a ten-year experience

Abstract

Autoimmune haemolytic anaemia (AHA) is one of the commonest autoimmune disorders of man. It is characterized by the binding of anti-erythrocyte autoantibodies to red blood cells and destruction of the coated cells in the reticuloendothelial system. Autoimmune disorders are said to be rare in indigenous African population, probably due to the widespread infectious diseases, which impair host's T-cell immunity. This study is therefore aimed at investigating the pattern of presentation and management outcome of patients with AHA seen over a period of 10 years (June 1988 to May 1998) at the Obafemi Awolowo University Teaching Hospitals Complex, lle-lfe. We retrospectively analyzed the records of patients with respect to the clinical, haematological, biochemical and serological features of AHA seen within the study period. Diagnosis was based on laboratory features of haemolytic anaemia and/or a positive direct anti-human globulin (Coombs’) test after excluding other causes of haemolytic anaemia. Treatment protocol and outcome were noted in all cases. We identified 13 patients with AHA (7 females, 6 males) aged 6 - 70 (median, 42) years. Six (42%) had secondary AHA and the remaining 8 presented with primary (idiopathic) AHA. Laboratory evidence of haemolysis (bone marrow erythroid hyperplasia and hyperbilirubinaemia) was found in all cases, while the direct Coomb's test was positive in 10 (76.9%) cases. All the patients had moderate-severe anaemia within the course of the disease, requiring blood transfusion. Remission was induced with prednisolone in all except three cases with secondary AHA who died of the primary disease before AHA could be controlled. Follow-up period post-remission ranged between 1 and 78 months. However, 2 (20%) are still being followed-up till the time of this report. This study agrees with the view that autoimmune disorders are not common in Nigerians, as documented for other Africans. It also shows that steroid therapy (prednisolone) is quite effective, especially, in idiopathic AHA, and that red cell transfusion could be useful in life-threatening anaemia.