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.