Complement haemolytic activity, circulating immune complexes and the morbidity of sickle cell anaemia

The aim of this study was to find out if the number of crises and complications of sickle cell anaemia (SCA) relate to complement function, or the levels of circulating immune complexes (CIC), complement factor B (Bf), C3 and C4. In 73 steady-state HbSS patients and 50 HbAA control subjects, we determined the haemolytic activity of the alternative pathway of complement (AP50), of the classical pathway (CH50); and the serum concentrations of Bf, C3, C4 and CIC. By clinical examination of each patient and review of the medical records, we determined the number of complications of SCA which had occurred and the mean number of crises per year over a minimum period of 3 years. The mean+/-SD AP50 for the patients (14+/-2 U/ml) was significantly lower than the control value of 16+/-3 U/ml (p<0.001). AP50 had a significant inverse correlation with the number of crises (r=-0.30, p<0.02). Mean+/-SD CIC in patients (0.45+/-0.38 g/l) was significantly higher than in controls: 0.24+/-0.15 g/l (p<0.002). CIC showed a significant direct correlation with the number of complications of SCA (r= +0.28, p<0.02). Mean+/-SD Bf in SCA patients (0.19+/-0.09) was higher than in controls (0.17+/-0.05). The difference reached marginal statistical significance (p=0.049). SCA patients and controls had no significant differences in CH50, C3 and C4. These parameters and Bf did not correlate with either the number of crises or complications. The mechanisms underlying the correlations observed in this study are yet to be fully elucidated.