[Delayed Hemolytic Anemia Associated with Anti-Jk3 Antibody Following Cardiovascular Surgery;Report of a Case].

: We report a 73-year-old man who underwent total aortic arch replacement with an open stent graft for aortic arch aneurysm. The patient received blood transfusion intraoperatively without any signs of hemolysis. However, on post-operative day 16, he developed hemoglobinuria, and was found to have anemia, elevated serum lactate dehydrogenase, elevated total bilirubin, and decreased serum haptoglobin. Initially, the cause for these findings was unknown. Upon further testing, however, antibodies against the high frequency antigen, anti-Jk3 was identified. The patient was conservatively treated and was discharged in stable condition on post-operative day 24. Jka and/or Jkb are red blood cell antigens observed in over 99.9% of the population worldwide. Jk (a-b-) represents the null phenotype and is very rare. This specific phenotype can be extremely dangerous, as homologous transfusion might cause severe delayed hemolysis. The actual patient carried Jk (a-b-) phenotype and, due to intraoperative transfusion of red blood cells, anti-Jk3 antibody was produced. Although very rare, patients who carry the red blood cell phenotype Jk( a-b-) may experience dangerous, or even fatal outcome due to such a common medical treatment as blood transfusion, if this condition is not recognized beforehand.