IgA Anti-red Blood Cell Auto-antibodies in Evans Syndrome

In Evans syndrome, IgG auto-antibodies (Abs) and/or complement components are frequently detected on red blood cells (RBC) in the direct antiglobulin test (DAT). A 70-year-old man with Evans syndrome diagnosed four years previously presented with a persistent autoimmune haemolytic anaemia, despite immunosuppressive treatment and normalization of platelet count. The RBC allo- and auto-Abs screening and identification were performed by indirect antiglobulin test (IAT) and DAT. In March 2006, no circulating anti-RBC auto-Abs were found in IAT but the DAT was positive with anti-IgG (++), -C3d (weak) and -IgA (++). Follow up for 11 months revealed anti-RBC IgA auto-Abs on five out of six samples. IAT was positive for RBC auto-Abs on three samples. No correlation between the haemoglobin level and the strength of reactivity of IgG and IgA auto-Abs was observed. IgA anti-RBC auto-Abs are present in Evans syndrome. To detect these Abs and characterize their role, DAT procedures should systematically include anti-IgA.