Hemolytic Anemia and Pancytopenia in Glutathione Reductase Deficiency: Further Experience with Riboflavin

Abstract

Three cases of hemolytic anemia and pancytopenia with glutathione reductase (GR) deficiency are described. Since flavin adenine dinucleotide is the prosthetic group of GR, riboflavin was administered in order to increase the enzyme activity. Despite the return of the GR activity to normal values, the hemolytic anemia and pancytopenia did not improve. It is conceivable that our cases might represent a latent form of leukemia.

Adaptation of Red Cell Enzymes and Intermediates in Metabolic Disorders

“…6.4. 2), measured spectrophotometrically at 366 nm and 37 °C in triethanolamine buffer pH 7.6 (50 mmol/1 with 50 mmol/1 EDTA) using the standard methods as described previously [2,8,17,26,28]. Glutathione in stability was estimated using the technique of Beutler et al [7].…”

Riboflavin Status in a Saudi Population – A Study in Riyadh

“…It contains flavin adenine dinucleotide (FAD) as a pros- thetic group and its activity is affected by dietary riboflavin status. Riboflavin defi ciency produces GR deficiency [Schroter, 1969;Goebel and Goebel, 1972], while ad ministration of 5 mg/day of riboflavin has been shown to increase the GR activity [Beutler, 1969a-c], The degree of stimula tion of erythrocyte GR in vitro in the pres ence of FAD has been used to calculate the 'activity coefficient' (AC):…”

Haemolytic Anaemia with Hereditary Pyruvate Kinase Instability Developing Acute Leukaemia

“…The clinical course of this woman showed a conversion of pancytopenia as a preleukaemic state into acute monocytic leukaemia with severe anaemia and thrombocytopenic haemorrhagic diathesis, finally causing death. Several reports are concerned with the connection between acquired red cell enzyme deficiencies, metabolic disorders and myeloproliferative diseases (Goswitz et a1 1966, Najman et a1 1969, Boivin et a1 1970, Schroter 1970, Goebel & Goebel 1972, Hakim et a1 1972, Blume et a1 1973b, Selroos & Vuopio 1973. Reduced enzyme rates of the glycolysis could be shown in experimental neoplastic tissues of the rat (Farron 1972).…”

“…Since several biochemical and microscopic investigations have demonstrated a coincidence of haemolytic anaemia, pancytopenia and red cell enzymopathies, possibly representing a preleukaemic state (Najman et a1 1969, Schroter 1970, Goebel 1972, further information has been furnished concerning the relation between myelofibrosis and acquired red cell enzyme deficiencies (Najman et a1 1969, Boivin et a1 1970, B l u e et a1 1973, Selroos & Vuopio 1973. The underlying cause of the increased red cell destruction in myelofibrosis is con-sidered, for instance to be localized in an enlarged spleen or it could perhaps be due to a PNH-like haemolytic mechanism with a membrane defect.…”