Nishimoto Takayuki, Bonkohara Yukihiro, Iijima Masaki
Department of Cardiovascular Surgery, National Hospital Organization Yokohama Medical Center, Yokohama, Japan.
Kyobu Geka. 2017 Jun;70(6):457-460.
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.
我们报告了一名73岁男性，其因主动脉弓动脉瘤接受了开放性支架移植物全主动脉弓置换术。患者术中接受输血，未出现任何溶血迹象。然而，术后第16天，他出现血红蛋白尿，并被发现患有贫血、血清乳酸脱氢酶升高、总胆红素升高和血清触珠蛋白降低。最初，这些发现的原因不明。然而，经过进一步检测，发现了针对高频抗原抗Jk3的抗体。患者接受保守治疗，术后第24天病情稳定出院。Jka和/或Jkb是全球超过99.9%的人群中观察到的红细胞抗原。Jk（a-b-）代表无效表型，非常罕见。这种特殊表型可能极其危险，因为同源输血可能导致严重的延迟性溶血。实际患者携带Jk（a-b-）表型，由于术中输注红细胞，产生了抗Jk3抗体。虽然非常罕见，但如果事先未识别出这种情况，携带红细胞表型Jk（a-b-）的患者可能会因输血这种常见医疗行为而经历危险甚至致命的后果。
