Anti-Km in a transfused man with McLeod syndrome

An 18 year old man has red cells of the McLeod phenotype in the Kell system but does not have chronic granulomatous disease. His red cells show acanthocytic morphology and there is evidence of a compensated hemolytic state. Following an accident he was transfused with blood of common Kell type and made anti-K and anti-Km. Leukocytes from the patient are deficient in Kx antigen but the cells appear to have normal function. The mother is a carrier of the variant Xk allele and has a double red-cell populatio of McLeod cells and cells of common Kell type. The family may be informative with respect to linkage between the Xk and Xg loci. One of the mother's 5 brothers and one of her maternal uncles are possible Xk:Xg recombinants.