An Inherited RBC Characteristic, NOR, Resulting in Erythrocyte Polyagglutination

Abstract

This report describes a new inheritable red blood cell characteristic, termed NOR, that is associated with polyagglutination. The NOR factor was shown to be inherited in an apparent dominant manner by 5 members of a two-generation family. NOR was clearly demonstrated to be a unique form of polyagglutination distinguishable from previously reported forms by serological tests using seed lectins, snail agglutinins, enzymes and absorption techniques. The NOR factor was characterized by the lack of reactivity with various seed and snail lectins, enhanced serum agglutinability after RBC proteolytic enzyme treatment, and the ability of hydatid cyst fluid to inhibit the serum hemagglutination reaction.

The differential expression of the blood group P1‐A4GALT and P2‐A4GALT alleles is stimulated by the transcription factor early growth response 1

“…In addition to P1, another two antigens, P k (also known as Gb3 and CD77) and NOR, have been included in the P1PK system. The P1, P k , and NOR are carbohydrate antigens and are carried on the glycosphingolipids of the cell membrane.…”

Human genetic disorders of sphingolipid biosynthesis

“…NOR-positive individuals apparently do not show any morbid symptoms, but their erythrocytes are agglutinated by most human sera, creating a risk of complications during transfusion of NOR erythrocytes. So far, the rare NOR polyagglutination syndrome has only been described in two families (Harris et al 1982;Kusnierz-Alejska et al 1999).…”

International Society of Blood Transfusion Working Party on red cell immunogenetics and blood group terminology: Cancun report (2012)

“…Thus, in addition to utilizing lactosylceramide for P k synthesis and paragloboside to make P1, this enzyme can also use globoside (P antigen) to synthesize NOR in the presence of the genetic alteration described above. NOR is a low‐prevalence antigen that was first described in an American family as an inherited polyagglutinable characteristic . Unlike other forms of polyagglutinable factors, NOR+ red blood cells (RBCs) are not agglutinated by the common plant lectins Arachis hypogea , Glycine soja , Salvia sclarea , Salvia horminum or Bandereia simplicifolia II, or by the snail lectin Helix pomatia .…”

“…Unlike other forms of polyagglutinable factors, NOR+ red blood cells (RBCs) are not agglutinated by the common plant lectins Arachis hypogea , Glycine soja , Salvia sclarea , Salvia horminum or Bandereia simplicifolia II, or by the snail lectin Helix pomatia . However, NOR+ RBCs are reactive with approximately 75% of ABO‐compatible human sera, and this reactivity was completely inhibited by hydatid cyst fluid or by avian P1 blood group substance .…”