Autoimmune haemolytic anaemia at Chris Hani Baragwananth academic hospital a retrospective study

Abstract

Background: Autoimmune haemolytic anemia (AIHA) is a rare, acquired haemolytic anaemia, in which auto-antibodies are produced against the red blood cell surface antigens, leading to increased destruction of these antibody coated red blood cells Aims of the study: To describe the clinical presentation, laboratory features, treatment and outcome of patients with AIHA, seen at Chris Hani Baragwanath Academic Hospital (CHBAH), during the period 01/01/2000 to 31/12/2012 (i.e. 13 years) Patients and Methods: This is a retrospective study conducted at CHBAH. The demographic, clinical, laboratory, treatment and outcome data were captured and analyzed on all eligible patients with AIHA Results: Fifty one (51) patients with AIHA were reviewed during this 13 year period. There were 40 females and 11 males, with a female to male ratio of 3.63:1. The median age of the patients was 36 years (range 14 – 74 years). Symptomatic anaemia was present in all the patients (100%). The mean haemoglobin at presentation was 4.84 g/dl range (1.5-10.3 g/dl) and the mean MCV (mean cell volume) was 108 fl (range 90.7-128 fl). Jaundice was noted in 75% of the patients, while splenomegaly was evident in 29% of the patients. Fever was present in 12% of the patients. A secondary cause was identified in 66% of the patients. The most common secondary cause was HIV (human immunodeficiency virus) infection. In the remaining 34% of the patients, the aetiology was unknown (idiopathic). In addition to supportive measures (such as blood transfusion), corticosteroids formed the cornerstone of treatment. The increment of the haemoglobin at 3 weeks was a mean of 3 g/dl. A more rapid response was seen in primary compared to secondary AIHA. Conclusion: AIHA is a rare condition, which may be idiopathic (primary) or secondary. Approximately 4-5 new patients are seen each year. At CHBAH, AIHA presents at a younger age (approximately one decade earlier) with a marked female predominance. Secondary causes are more common than idiopathic AIHA, with HIV being the dominant secondary cause. The management includes supportive measures (blood transfusion) and specific treatment modalities, such as immunosuppressives (with corticosteroids being the most commonly used agent), monoclonal antibodies and splenectomy.