The metabolitic role of the iron porphyrin complex in the development of anemic syndrome in patients with minor form of β-thalassemia

Abstract

Aim. To determine the significance of the main indicators of metabolism of porphyrins and markers of iron metabolism in the development of anemic syndrome during β-thalassemia minor. Methods. Examined were 58 patients with β-thalassemia minor with a concomitant deficiency of the enzyme glucose-6-phosphate dehydrogenase and 150 patients β-thalassemia minor without the concomitant enzymopathy. Hemoglobin electrophoresis was used to verify the diagnosis of β-thalassemia. Evaluated were the main indicators of porphyrin metabolism in the erythrocytes and urine in comparison with the reserve pool of iron in the blood serum, bone marrow and liver. Results. It has been shown that the assessment of soluble transferrin receptors and zinc protoporphyrin - is an alternative to assessing serum iron. Changes in the metabolism of iron is not the only cause of hypochromic microcytic anemia in patients with β-thalassemia minor. A large role is played by the fluctuations of the concentration of free forms of porphyrins in erythrocytes. With their help developed were differential diagnostic criteria, which distinguish the minor form from other forms of β-thalassemia and other iron deficiency states. During β-thalassemia minor, regardless of concomitant enzymopathy present was the instability of the main indicators of metabolism of the iron porphyrin complex. Conclusion. Extended evaluation of indicators of iron metabolism in conjunction with the assessment of precursors of porphyrin metabolism and free erythrocyte porphyrins is of high diagnostic value in determining the severity of anemia.

Keywords

β-thalassemia minor / glucose-6-phosphate dehydrogenase / uroporphyrin / coproporphyrin / protoporphyrin / porphobilinogen / zinc protoporphyrin / serum iron

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RIGHTS & PERMISSIONS

Baytaeva D.A., Bessmeltsev S.S.