Transfusion support for a patient with McLeod phenotype without chronic granulomatous disease and with antibodies to Kx and Km

Background and Objectives  Kell antigens are encoded by the KEL gene on the long arm of chromosome 7. Kx antigen is encoded by the XK gene on the short arm of the X chromosome. Kell and Kx proteins in the red cell membrane are covalently linked by a disulphide bond. The McLeod phenotype is characterized by weakened expression of antigens in the Kell blood group system, absence of Km and Kx antigens, and acanthocytosis. It has an X‐linked mode of inheritance with transmission through carrier females. Some males with the McLeod syndrome also have chronic granulomatous disease (CGD). It is generally believed that patients with non‐CGD McLeod may develop anti‐Km but not anti‐Kx, but that those with CGD McLeod can develop both anti‐Km and anti‐Kx.

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