Dapsone-associated Heinz body hemolytic anemia in a Cambodian woman with hemoglobin E trait.

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.

Talassemia e hanseniase / Thalassemia and leprosy

A talassemia B foi investigada em 165 brasileiros descendentes nao miscigenados, 80 dos quais eram doentes de hanseniase, da forma virchowiana, e 85 estudantes universitarios (grupo controle). A frequencia da talassemia foi de 6,25 por cento entre hansenianos e de 5,88 por cento entre os estudantes. Apesar da similaridade da distribui;ao geografica tanto da hanseniase quanto do gene da talassemia B eram algumas regioes do globo, sobretudo da Asia, a frequencia de individuos, com o estigma talassemico observada nas amostras analisadas nao apoia a hipotese de que a hanseniase possa ter contribuido para manter as altas frequencias desse alelo, por intermedio da sele;ao a favor dos talassemicos heterozigotos.

[Frequency of hemoglobin abnormalities and hemoglobinopathies in Mozambique peoples (comparison between leprosy and non-leprosy subjects]. / Uber die Häufigkeit von Hämoglobinanomalien und Hämoglobinopathien bei moçambiquanischen Völkerschaften. (Mit Vergleich zwischen Leprösen und Nicht-Leprösen)

In 588 bloodsamples of negride natives from Moçambique, preferably Chuabo and Macua, haemoglobin analyses were performed. In 21 cases an increase of Hb A2 was found, indicating the presence of heterozygous beta-thalassaemia, in one case the changes in Hb-analysis were typical for beta-delta-thalassaemia, 18 samples could be shown to contain Hb S, typical for the heterozygous sickle cell trait. Futhermore in 7 cases Hb A2' was found. In two bloodsamples haemoglobin variants were observed, which according to their electrophoretical mobility were assumed to represent Hb D in one case, and Hb G in the other. In the Chuabo population the frequency of the thalassaemia gene was found to be more than twice as high as in the Macua population. In non-lepers Hb S was observed with a remarkable higher incidence than in lepers.