e. An interesting association between the Kell system and an X-linked disease
of childhood, chronic granulomatous disease (CGD), has been described. In this
disease, the neutrophils can phagocytize but not kill certain pathogenic organisms.
Normal neutrophils and monocytes do not possess any of the antigens that are products
of the Kell locus but they do possess the Kx antigen. Leukocytes from boys with CGD
have been shown to lack the Kx determinant. Most cases of CGD have red blood cells
of common Kell phenotype but a few cases have the McLeod phenotype, and run the
risk of forming anti-KL + Kx if transfused with blood of common Kell type.
f. Antibodies in the Kell system usually react optimally by the anti-globulin test
(AGT). Table 2-20 shows the usual serologic behavior of the antibodies. The
antibodies have been responsible for both fatal transfusion reactions and severe
hemolytic disease of the newborn.
g. The K antigen is the most immunogenic after Rho(D). Fortunately, 90 serious
problems... Similarly, finding compatible blood for patients with anti-Kpa, -Jsa, -Kw(K8), -
UIa(K10), or -K17 presents no real difficulty and is well within the capability of any blood
Bank; however, finding blood for a patient with an antibody directed against one of the
high-frequency antigens k, Kpb, Jsb, Ku, KL, K11-K16, or K18 is an extremely difficult
task and is unlikely to be accomplished by screening several hundred random donors.
Table 2-20. Serologic behavior of Kell system antibodies.