At-a is a high-frequency red blood cell (RBC) antigen. Anti-At-a has b
een reported in rare At(a-) black subjects. We report two cases of ant
i-At-a. A clinically significant anti-At-a was found in a 26-year-old
black woman with systemic lupus erythematosus. The patient had a trans
fusion reaction with chills and nausea during a RBC survival study, an
d 95% of the radiolabeled At(a+) RBCs were destroyed within 3 h. A con
currently performed monocyte monolayer assay was strongly reactive. An
ti-At-a thus can cause rapid hemolysis of transfused RBCs, but At(a-)
donor units are extremely scarce in rare donor registries. A second pa
tient at our hospital had anti-At-a which did not affect her newborn.
She also had autoimmune disease, insulin-dependent diabetes mellitus.