Low-incidence antigens can be defined as those which are known to occur in less than 1% of individuals in most populations. The red cells of almost all blood recipients or prenatal patients therefore lack these rare antigens meaning a chance exposure to blood which does carry a lowincidence antigen almost always results in potential alloimmunization. However, since the antigens are so rare, the chance of exposure is very low. Incompatibility because of an antibody to a low-incidence antigen therefore does not happen very often, but when it does, can cause the same clinical problems, transfusion reactions, hemolytic disease of the fetus and newborn (HDFN), as antibodies to more common specificities. Table 1 lists the low-incidence antigens currently recognized by the International Society of Blood Transfusion Committee on Terminology for Red Cell Surface Antigens. Most have been shown by biochemical and/or molecular methods to be rare variations in established blood group systems as shown. The antigens listed in the 700 series are recognized as distinct low-incidence antigens but there is no biochemical or genetic data that proves they belong to any of the blood group systems. Molecular studies have shown that most of the antigens are the result of a single nucleotide mutation in the gene which codes for a blood group protein, which in turn leads to a change of only one amino acid in the protein. In some cases the antigenic basis is more difficult to determine, particularly in the Rh, MNS and Gerbich blood group systems, where hybrid genes may be involved. For further information on the molecular basis of antigens the interested reader should consult a comprehensive textbook [1–3] Low-incidence antigens are categorized by their low frequency in most populations. Usually this means they are found to be rare or absent in all populations studied but in some cases the frequency can be much higher in some races or ethnic groups. One of the most striking examples of this is the Di antigen. It is rare on red blood cells from Caucasians and Africans but occurs in approxi-