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Effect of Primaquine on Erythrocytes with NADH‐Methaemoglobin Reductase Deficiency and Low Glutathione Reductase Activity
Author(s) -
Ajmar Franco,
Gaetani Gianfranco,
Garré Cecilia,
Bianchi Giovanna,
Salvidio Emanuele
Publication year - 1972
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1972.tb08880.x
Subject(s) - methemoglobin , primaquine , methylene blue , haemolysis , methemoglobinemia , glutathione reductase , glutathione , reductase , hemoglobin , chemistry , medicine , endocrinology , biochemistry , enzyme , glutathione peroxidase , immunology , photocatalysis , chloroquine , malaria , catalysis
Summary. Two young patients with congenital methaemoglobinaemia due to NADH‐methaemoglobin reductase deficiency associated with low activity of erythrocytic glutathione reductase (GSSG‐R) are described. Their red cells, labelled with 51 Cr and transfused into normal recipients, were susceptible to haemolysis by primaquine with an abnormal pattern of destruction. The administration of riboflavin raised the level of erythrocyte GSSG‐R, but did not alter the level of methaemoglobin. The administration of methylene blue not only reduced the methaemoglobin to almost normal levels, but also increased the activity of GSSG‐R. During methylene blue therapy, their red cells, tagged with 51 Cr and transfused into a normal recipient who was receiving methylene blue, were not susceptible to haemolysis by primaquine. Some possible reasons for the frequent association between NADH‐methaemoglobin reductase deficiency and low activity of GSSG‐R are discussed.