An Evans Syndrome Case Expressing Anti-Jksupa/supAutoantibody under Condition of Primary IgA Immunodeficiency

摘要

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 co-occurrence of autoimmune haemolytic anaemia and primary IgA immunodeficiency syndrome, analysis focusing on specificity for red blood cells antigens will be required in IgA immunodefi-ciency syndrome patients.