Cold Agglutinin Disease due to Anti-Pr Cold Autoantibody in a Patient with Bone Marrow Transplantation / 대한수혈학회지

ABSTRACT

Cold agglutinin disease (CAD) is a most common autoimmune hemolytic anemia (AIHA) induced by cold antibody. CAD represents approximately 16-32% of AIHA cases and causative cold autoantibodies commonly show specificity against the I antigen. We report a case of cold agglutinin disease with anti-Pr cold autohemolysin. A 20 year old woman with a history of bone marrow transplantation was admitted with nausea, vomiting, and pallor. Direct antiglobulin tests were positive with IgG and C3d specific AHG reagents. Cold agglutinin titer was as high as 11024 at 4degrees C, 116 at room temperature, negative at 37degrees C. The agglutinin titer was diminished after treatment with protease, ficin and immunohematologic results of cold agglutinin was compatible with anti-Pr specificity. In unexpected antibody identification test, anti-M which showed reactivity at anti-human globulin phase was identified. Washed and prewarmed 16 units of A+, M antigen negative red blood cells were transfused. After two weeks, patient was improved with steroid therapy and experienced relief of fever and hemolysis, and she was discharged.