Evans syndrome in a pediatric liver transplant recipient with an autoantibody with apparent specificity for the KEL4 (Kpb) antigen  

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Immunohematology

American National Red Cross

Subject: Medical Laboratory Technology

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ISSN: 0894-203X
eISSN: 1930-3955

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VOLUME 30 , ISSUE 1 (March 2014) > List of articles

Evans syndrome in a pediatric liver transplant recipient with an autoantibody with apparent specificity for the KEL4 (Kpb) antigen  

Scott A. Koepsell / Kerry Burright-Hittner / James D. Landmark

Keywords : autoimmune hemolytic anemia, Kell blood group, Evans syndrome

Citation Information : Immunohematology. Volume 30, Issue 1, Pages 14-17, DOI: https://doi.org/10.21307/immunohematology-2019-093

License : (Transfer of Copyright)

Published Online: 29-October-2019

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ABSTRACT

Although most warm red blood cell (RBC) autoantibodies react broadly with panel cells in addition to the patient’s own RBCs, occasionally an autoantibody with specificity for a specific blood group antigen is encountered. Rare cases of warm autoantibodies with specificity for the Kpb antigen of the Kell blood group system have been described. We report a pediatric transplant recipient with anemia, immune-mediated hemolysis, thrombocytopenia, and a warm autoantibody with apparent anti-Kpb specificity. The patient’s autoimmune anemia and thrombocytopenia responded well to discontinuing the immunosuppressant tacrolimus, transfusions with Kp(b–) RBCs, and intravenous immunoglobulin therapy, with disappearance of the pathologic antibody. During the autoimmune hemolysis, the patient’s RBCs did not react with antisera specific for Kpb. However, repeat testing of the patient’s RBCs with Kpb-specific antisera 15 months after the resolution of hemolysis showed reactivity, indicating that the RBC autoantibody was associated with a transient disappearance of the Kpb antigen.

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