Lachant N A, Tanaka K R
Department of Medicine, Harbor-UCLA Medical Center, UCLA School of Medicine, Torrance 90502.
Am J Med Sci. 1987 Nov;294(5):364-8. doi: 10.1097/00000441-198711000-00012.
A Cambodian woman with hemoglobin E trait (AE) and leprosy developed a Heinz body hemolytic anemia while taking a dose of dapsone (50 mg/day) not usually associated with clinical hemolysis. Her red blood cells (RBCs) had increased incubated Heinz body formation, decreased reduced glutathione (GSH), and decreased GSH stability. The pentose phosphate shunt activity of the dapsone-exposed AE RBCs was increased compared to normal RBCs. Although the AE RBCs from an individual not taking dapsone had increased incubated Heinz body formation, the GSH content and GSH stability were normal. The pentose phosphate shunt activity of the non-dapsone-exposed AE RBCs was decreased compared to normal RBCs. Thus, AE RBCs appear to have an increased sensitivity to oxidant stress both in vitro and in vivo, since dapsone does not cause hemolytic anemia at this dose in hematologically normal individuals. Given the influx of Southeast Asians into the United States, oxidant medications should be used with caution, especially if an infection is present, in individuals of ethnic backgrounds that have an increased prevalence of hemoglobin E.
一名患有血红蛋白E性状（AE）的柬埔寨妇女同时患有麻风病，在服用通常不会引起临床溶血的剂量的氨苯砜（50毫克/天）时，发生了海因茨小体溶血性贫血。她的红细胞（RBC）孵育后海因茨小体形成增加，还原型谷胱甘肽（GSH）减少，GSH稳定性降低。与正常RBC相比，暴露于氨苯砜的AE RBC的磷酸戊糖途径活性增加。虽然未服用氨苯砜的个体的AE RBC孵育后海因茨小体形成增加，但GSH含量和GSH稳定性正常。与正常RBC相比，未暴露于氨苯砜的AE RBC的磷酸戊糖途径活性降低。因此，AE RBC在体外和体内似乎对氧化应激更敏感，因为氨苯砜在这个剂量下不会使血液学正常的个体发生溶血性贫血。鉴于东南亚人不断涌入美国，对于血红蛋白E患病率增加的种族背景个体，使用氧化药物时应谨慎，尤其是存在感染时。
