A Guide to Terminology for Rh Immunoprophylaxis.

Rh immunoprophylaxis for Rh-negative women requires an understanding of terminology used for Rh blood typing laboratory reports. The pathophysiology of Rh hemolytic disease of the fetus and newborn was elucidated by studies in rhesus monkeys. Subsequent studies revealed that the human blood group antigen responsible for Rh hemolytic disease of the newborn (D antigen) is related to, but different from, the rhesus monkey antigen. Weak expression of the D antigen on red cells, originally termed D, is currently reported by laboratories as a "serologic weak D phenotype," which can be further defined by RHD genotyping to be either a weak D type or a partial D phenotype. Weak D types 1, 2, or 3 are molecularly defined RHD weak D types, which have an adequate number of intact D antigens to be managed safely as Rh-positive. Partial D phenotypes result from mutations causing loss of one or more D epitopes. Most persons with a partial D phenotype have sufficient D antigen to type as Rh-positive. Some women with a partial D phenotype are detected as serologic weak D phenotypes by routine Rh typing. Whether they type as Rh-positive or serologic weak D phenotype, after being exposed to Rh-positive red cells by transfusion or pregnancy, women with partial D phenotype can form anti-D antibodies and, if they do, are at risk for hemolytic disease of the fetus and newborn. A pregnant woman with a laboratory report of a serologic weak D phenotype should be further tested for her RHD genotype to resolve whether her case should be managed as Rh-positive or Rh-negative. For more than five decades, the practice of Rh immunoprophylaxis has remained unchanged in terms of the dose of Rh immune globulin and timing of injections. In contrast, advances in the science of Rh blood typing have resulted in a continuously evolving terminology, obliging obstetricians to update their vocabulary to guide their practice. The following review and glossary provide guidance for current Rh terminology and the rationale for changes.