Altered glycosylation leads to Tr polyagglutination.
BACKGROUND
Polyagglutination refers to red blood cells (RBCs) that are agglutinated by a high proportion of ABO-matched adult sera but not by cord sera. Polyagglutinable RBCs have been associated with microbial infection, myeloproliferative disorders, and myelodysplasia. Lectins aid in the identification of polyagglutination.


METHODS
A Hispanic male infant with mild hemolytic anemia, a "Bernard-Soulier-like" syndrome, intermittent neutropenia, mitral valve regurgitation, ligament hyperlaxity, and mild mental retardation was studied. The patient's Group O RBCs were polyagglutinable; they were agglutinated by normal human sera, several lectins [including Arachis hypogea, Salvia sclarea, Salvia horminum, Glycine max, Ulex europaeus, Griffonia simplicifolia I, and Gr. simplicifolia II], and some monoclonal antibodies. His RBCs were not agglutinated by cord sera, Dolichos biflorus, or Phaseolus lunatus. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis on the RBC membranes followed by staining with periodic acid-Schiff stain showed markedly reduced staining of glycophorins A and B. Staining with Coomassie brilliant blue revealed that Band 3 has a faster mobility than normal.


CONCLUSIONS
Collectively, the results suggest that the patient's RBCs have a reduction in N-acetylneuraminic acid on both N- and O-glycans, exposing, respectively, beta1,4-galactosidase and beta1,3-galactosidase. The patient likely has an altered glycosyltransferase that results in defective glycosylation in RBCs and other cell lineages. This type of polyagglutination was named Tr.
Halverson
G R
GR
From the New York Blood Center, New York, New York.
Lee
A H
AH
Øyen
R
R
Reiss
R F
RF
Hurlet-Jensen
A
A
Reid
M E
ME
D000743:Anemia, Hemolytic / Q000150:complications; D001606:Bernard-Soulier Syndrome / Q000150:complications; D001769:Blood; D004591:Electrophoresis, Polyacrylamide Gel; D004912:Erythrocytes / Q000737:chemistry*; D005312:Fetal Blood; D006031:Glycosylation; D006384:Hemagglutination / Q000235:genetics; D006402:Hematologic Diseases / Q000097:blood* / Q000150:complications; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000150:complications; D037102:Lectins; D008297:Male; D008944:Mitral Valve Insufficiency / Q000150:complications / Q000601:surgery; D019158:N-Acetylneuraminic Acid / Q000097:blood; D009503:Neutropenia / Q000150:complications
D037102:Lectins; D019158:N-Acetylneuraminic Acid