An Evans Syndrome Case Expressing Anti-Jka Autoantibody under Condition of Primary IgA Immunodeficiency

Autoimmune haemolytic anaemia is a haemolytic disease resulting from an autoimmune reaction to the surface of red blood cells. A part of autoantibody is known to react with the blood type antigen. This is the case of a 14 years old female with Evans syndrome in which autoimmune haemolysis may cause from anti-Jka autoantibody reaction. As this case is complicated with primary IgA immunodeficiency syndrome, anti-Jka autoantibody may occur under the condition of primary immunodeficiency status, in which autoantibody production is accelerated. Considering the cooccurrence of autoimmune haemolytic anaemia and primary IgA immunodeficiency syndrome, analysis focusing on specificity for red blood cells antigens will be required in IgA immunodeficiency syndrome patients.