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- Title
Altered glycosylation leads to Tr polyagglutination.
- Authors
Halverson, G.R.; Lee, A.H.; Øyen, R.; Reiss, R. F.; Hurlet-Jensen, A.; Reid, M. E.
- Abstract
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.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[includingArachis hypogea,Salvia sclarea,Salvia horminum,Glycine max,Ulex europaeus,Griffonia simplicifolia I, andGr. simplicifolia II], and some monoclonal antibodies. His RBCs were not agglutinated by cord sera,Dolichos biflorus,orPhaseolus 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.Collectively, the results suggest that the patient's RBCs have a reduction inN-acetylneuraminic acid on both N- and O-glycans, exposing, respectively,β1,4-galactosidase andβ1,3-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.
- Subjects
AGGLUTINATION; ANTIGEN-antibody reactions; IMMUNOGLOBULINS; BLOOD plasma; HEMAGGLUTININ; LECTINS; HEMOLYTIC anemia; MITRAL valve
- Publication
Transfusion, 2004, Vol 44, Issue 11, p1588
- ISSN
0041-1132
- Publication type
Article
- DOI
10.1111/j.0041-1132.2004.04133.x