title: First example of anti-Kx in a person with the McLeod phenotype and without chronic granulomatous disease.
abstract: BACKGROUND
  Kx is lacking in the RBCs of patients with the McLeod syndrome. This condition is sometimes associated with chronic granulomatous disease (CGD). If given allogeneic RBCs, CGD patients with the McLeod phenotype may produce anti-Kx and anti-Km, and only phenotypically matched McLeod blood would be compatible. McLeod phenotype persons without CGD have made anti-Km but not anti-Kx (2 examples), and thus both McLeod and K(O) blood would be compatible.
  
  
  METHODS
  RBCs from a transfused patient with the McLeod phenotype but not with CGD (non-CGD McLeod) were typed for the Kell blood group antigens, and the plasma was analyzed for the presence of antibody by agglutination. The molecular basis was determined by analyzing for XK protein on RBC membranes by Western immunoblotting, by sequencing the XK gene, and by RFLP.
  
  
  RESULTS
  The RBCs did not react with anti-Kx + anti-Km and showed weakening of Kell system antigens. The patient's plasma reacted moderately (2+) with RBCs of common Kell type and strongly (4+) with K(O) RBCs and RBCs of common Kell type treated with dithiothreitol, and did not react with McLeod RBCs. XK protein was absent from the RBC membranes. The XK gene had a point mutation in the donor splice site of intron 1 (G>C).
  
  
  CONCLUSIONS
  This is the first report describing the molecular alteration in a non-CGD McLeod patient who has made anti-Kx. The immune response of people with the McLeod phenotype can vary, and K(O) blood may not always be compatible.
authors: New York Blood Center, New York, New York, USA.
authors: D C
authors: DC
authors: Russo
authors: R
authors: R
authors: Oyen
authors: V I
authors: VI
authors: Powell
authors: S
authors: S
authors: Perry
authors: J
authors: J
authors: Hitchcock
authors: C M
authors: CM
authors: Redman
authors: M E
authors: ME
authors: Reid
mesh_terms: D000917:Antibody Formation; D006105:Granulomatous Disease, Chronic / Q000097:blood*; D006801:Humans; D007518:Isoantibodies / Q000097:blood* / Q000276:immunology; D007626:Kell Blood-Group System / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012150:Polymorphism, Restriction Fragment Length
chemical_list: D007518:Isoantibodies; D007626:Kell Blood-Group System