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Kx Antigen, The McLeod Phenotype, and Chronic Granulomatous Disease: Further Studies 1
Author(s) -
Marsh W. L.,
ØYen Ragnhild,
Nichols Margaret E.
Publication year - 1976
Publication title -
vox sanguinis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.68
H-Index - 83
eISSN - 1423-0410
pISSN - 0042-9007
DOI - 10.1111/j.1423-0410.1976.tb04659.x
Subject(s) - chronic granulomatous disease , phenotype , antigen , immunology , medicine , biology , genetics , gene
. Leukocytes of nine unrelated boys with X‐linked chronic granulomatous disease lack Kx antigen. In three of these cases, the red cells also lack Kx and have the McLeod phenotype and abnormal morphology. X‐linked chronic granulomatous disease CGD can thus be separated into two types. Type I cases have an antigenic deficiency that is restricted to the phagocytic leukocytes while in type II, the deficiency involves both leukocytes and red cells. Red cells of type II CGD patients have enhanced i antigen activity suggesting that they are under hemopoietic stress. Normal Kx synthesis is directed by an X‐linked gene named X 1 k . Three rare variants, X 2 k, X 3 k , and X 4 k order the different permutations of leukocyte and red cell Kx antigen production that have been recognized.