01859nas a2200289 4500000000100000008004100001260001300042653002700055653001000082653002200092653001300114653001200127653002800139653001100167653001600178653001700194653001700211653002600228653001100254100001600265700001500281245009700296300001000393490000800403520114400411022001401555 1987 d c1987 Nov10a2,3-Diphosphoglycerate10aAdult10aAnemia, Hemolytic10aCambodia10aDapsone10aDiphosphoglyceric Acids10aFemale10aGlutathione10aHeinz Bodies10aHemoglobin E10aHemoglobins, Abnormal10aHumans1 aLachant N A1 aTanaka K R00aDapsone-associated Heinz body hemolytic anemia in a Cambodian woman with hemoglobin E trait. a364-80 v2943 a

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.

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