An Evans Syndrome Case Expressing Anti-Jk<sup>a</sup> Autoantibody under Condition of Primary IgA Immunodeficiency
- 1 Department of Hematology and Oncology, Kita-Harima Medical Center, Ono, Japan
- 2 Institute of Biomedical Research and Innovation, Kobe, Japan
- 3 Department of Blood Transfusion, Kobe University Hospital, Kobe, Japan
- 4 Department of Blood Transfusion, Kobe University Hospital, Kobe, Japan
- 5 Department of Pediatric Medicine, Kobe University Hospital, Kobe, Japan
- 6 Department of Pediatric Medicine, Kobe University Hospital, Kobe, Japan
- 7 Department of Pediatric Medicine, Kobe University Hospital, Kobe, Japan
- 8 Department of Pediatric Medicine, Kobe University Hospital, Kobe, Japan
- 9 Department of Pediatric Medicine, Kobe University Hospital, Kobe, Japan
- 10 Department of Pediatric Medicine, Kobe University Hospital, Kobe, Japan
- 11 Department of Blood Transfusion, Kobe University Hospital, Kobe, Japan
- 12 Department of Pediatric Medicine, Kobe University Hospital, Kobe, Japan
Abstract
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-Jk a autoantibody reaction. As this case is complicated with primary IgA immunodeficiency syndrome, anti-Jk a 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.
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