Immune hemolytic anemia associated with anti-Kell and a carrier state for chronic granulomatous disease.
A patient presented with immune hemolytic anemia associated with a strongly positive direct antiglobulin test (IgG and complement). Anti-K was eluted from the patient's red cells, which were shown to be K negative. A powerful complement-binding anti-K was present in the serum together with another antibody(ies) showing characteristics resembling anti-Bg. Leukocyte antibodies were also present in the patient's serum. The anti-K could be adsorbed and eluted from K negative red cells in vitro. It is suggested that either non-specific adsorption of the anti-K may have occurred due to the Matuhasi-Ogata phenomenon; or, the antibody was an auto "minicking anti-K" capable of reacting with a broader specificity within the Kell system. A serendipitous finding was that the patient was a carrier for chronic granulomatous disease. The associations of immune hemolytic anemia, chronic granulomatous disease, and the Kell system are discussed.
D000042:Absorption; D000327:Adsorption; D000373:Agglutinins; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000276:immunology*; D000918:Antibody Specificity; D001323:Autoantibodies; D001667:Binding, Competitive; D001789:Blood Group Antigens; D001792:Blood Platelets; D002353:Carrier State / Q000276:immunology*; D003080:Cold Temperature; D003298:Coombs Test; D003429:Cross Reactions; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006680:HLA Antigens; D006386:Hemagglutination Tests; D006801:Humans; D007626:Kell Blood-Group System; D008875:Middle Aged; D013997:Time Factors
D000373:Agglutinins; D001323:Autoantibodies; D001789:Blood Group Antigens; D006680:HLA Antigens; D007626:Kell Blood-Group System