The H blood group system

Abstract The H blood group system, ISBT symbol H (018), consists of a single antigen (H) defined by a terminal fucose residue found on red blood cells and in secretions formed by the action of α-1,2-fucosyltransferases 1 (α2FucT1) and 2 (α2FucT2), respectively. Mutant alleles of the corresponding FUT1 and FUT2 genes result in either a H– phenotype (Bombay phenotype, O h ) or a weak H phenotype (para-Bombay, H+ w ). In addition, the FUT2 gene is the molecular basis of the secretor (Se) status, and homozygosity or compound heterozygosity for null alleles is associated with the nonsecretor (se) status. H– individuals have natural anti-H (mostly IgM), which can cause severe hemolytic transfusion reactions with intravascular hemolysis. Immunohematology 2016;32:112–118.