Prognosis of hemolytic anemia in G6PD- subjects. Multifactorial cluster analysis of biochemical characteristics of red cell age groups.

Individual susceptibility of 10 G6PD- hemizygotes to oxidative hemolytic agents was tested on the basis of multifactorial cluster analysis of biochemical indices of erythrocyte populations; the indices related to G6PD activity and glucose metabolism were analyzed under physiological and oxidative stress conditions in very young, exactly adult and very old red cell suspensions. Biochemical images of G6PD- erythrocytes were obtained and compared with the donor (7 subjects) biochemical image on a IBM-PC computer according to a special "taxon" program. As a result, a stable subdivision of 10 Gd- biochemical images into 5 taxons was formed; each taxon included G6PD subjects with a certain form of clinical appearance of G6PD deficiency. Multifactorial cluster analysis of biochemical data on the erythrocyte population allows a clinical prognosis for G6PD- subjects.

New stable mutant (Gd(-) variants: G6PD Tashkent and G6PD Nucus. Molecular basis of hereditary enzyme deficiency.

This paper represents studies on the molecular basis of G6PD deficiency in erythrocytes of 4 hemizygote patients. In all cases G6PD deficiency was due to an abnormal kinetic and (or) physico-chemical characteristics of the mutant enzymes. Two of 4 variants were characterized as G6PD EL Fayoum like (unstable, class 2). The other two are new variants: G6PD Tashkent (low Ki by NADPH, class 3) and G6PD Nukus (Km for G6P 127 microM, class 2). For the new mutant Gd(-) stable variants the amount of G6PD in total red cell population found immunologically was similar to the normal level. Stimulation of the pentose phosphate pathway (PPP) of G6PD Nucus erythrocytes insignificantly increased the rate of glucose consumption while in the case of G6PD Tashkent methylene blue raised the rate of PPP to half of the maximal rate of stimulated normal red cells.