At(a-) phenotype: description of a family and reduced survival of At(a+) red cells in a proposita with anti-Ata.

摘要

BACKGROUND: There is a paucity of data on the August (At) blood group antigen and clinical significance of anti-Ata. STUDY DESIGN AND METHODS: A proposita with the At(a-) phenotype was identified by the finding of anti-Ata in the cord blood eluate of her fifth live infant. Family members were studied, and a small aliquot of 51Cr-labeled At(a+) red cells was transfused to determine survival. RESULTS: There was no evidence of hemolytic disease of the newborn, as determined by the normal hemoglobin and bilirubin and normal clinical conditions. Six of seven siblings were tested, and two At(a-) female siblings were identified. In contrast to the proposita, neither sister had detectable anti-Ata in her serum, although each has had only one pregnancy. A monocyte monolayer assay performed on serum from the proposita gave a result of 20-percent hemolysis (normal, <3%), which is consistent with a clinically significant antibody. Transfusion of a small volume of allogeneic red cells that were phenotypically matched with the proposita, except for Ata, resulted in a 1-hour survival of 95 percent, but a 24-hour survival of only 18 percent, of the transfused cells. The survival pattern was exponential, which is characteristic of a non-complement-binding IgG antibody. CONCLUSION: Despite the absence of hemolytic disease of the newborn, this example of anti-Ata would appear to be a clinically significant antibody for the purposes of transfusion practice. Therefore, approaches to the management of clinical situations in which transfusion is required or likely should focus on the availability of autologous cells or frozen allogeneic At(a-) red cells.