Influence of splenectomy on the properties of erythrocytes with hemoglobin Volga disease.

Hemoglobin Volga is a rare unstable hemoglobin in which there is a replacement of an internal alanine residue, beta 27 (B9), by an aspartate. From a clinical point of view it is characterized by a moderately severe Heinz body hemolytic anemia. A possible clue to the cause of the hemolysis is the increased vulnerability to oxidation of Hb Volga, with increased free radical turnover and consequent damage to the red cell membrane. Splenectomy performed on carriers of Hb Volga may have a positive outcome leading to improvements in both the clinical condition of the patient and hematological variables such as hemoglobin concentration and bilirubin concentration. With the aim of defining a more complete biochemical picture of the beneficial effect of splenectomy in this disease, we have evaluated some enzymic activities of the red cells of a young patient with Hb Volga disease, before and after splenectomy. In particular, we have investigated the activity of superoxide dismutase (superoxide: superoxide oxidoreductase), glutathione peroxidase (GSH peroxidase, glutathione: hydrogen-peroxidase oxidoreductase) and catalase (hydrogen-peroxide: hydrogen-peroxide oxidoreductase) that catalyze reactions relevant to the steady-state concentration of potentially toxic oxygen derivatives such as O2- and H2O2. Besides, we have carried out experiments on the erythrocytic membrane to evaluate eventual changes on the chemical (i.e. peroxidation) and physico-chemical (i.e. fluidity) properties following surgery.