Protective action of deferiprone and deferoxamine in erythrocytes isolated from patients with β-thalassemias

Abstract

One of the most deleterious consequences of iron overload in thalassemia is the presence of non-transferrin bound iron (NTBI), a free radical that acts as a catalyst for free oxygen radicals, in particular for hydroxyl free radicals (OH.). These radicals oxidize both membrane lipids and proteins causing irreversible damage to biologically important molecules and cellular structures. Treatment with iron chelators has been important to improve survival of these individuals. The aim of this work was the study on the effects of deferoxamine (DFO) and deferiprone (DFP) on erythrocytes under the pro-oxidative action of TBHP isolated from normal individuals and patients with ß-thalassemia. The in vitro action of deferoxamine and deferiprone on the oxidative metabolism of erythrocytes from ß-thalassemic patients treated at the Centro de Hematologia e Hemoterapia do Paraná (HEMEPAR), Brazil, under the pro-oxidative action of TBHP was studied. Methemoglobin concentrations, reduced glutathione (GSH), hemolysis indexes and the enzyme activities of G6-PD and GR were determined. The oxidation indexes were higher in erythrocytes of ß-thalassemic individuals than those from normal individuals. Treatment of the normal and ß-thalassemic erythrocytes with DFO and/or DFP protected against the formation of GSH promoted by TBHP. Rev. Bras. Hematol. Hemoter.

Deferiprone Reduces Amyloid-β and Tau Phosphorylation Levels but not Reactive Oxygen Species Generation in Hippocampus of Rabbits Fed a Cholesterol-Enriched Diet

“…Deferiprone protected cortical neurons and SHSY-5Y cells from ferric iron, hydrogen peroxide, MPP + , and Aβ-induced neuronal cell death [22]. Deferiprone also reduced atherogenesis in rabbits [23], oxidative stress in erythrocytes, platelets and polymorphonuclear leukocytes from myelodysplastic syndrome patients [24], suppressed experimental autoimmune encephalomyelitis and inhibited T-cell function in mice [25], and reversed glutathione depletion in erythrocytes isolated from patients with β-thalassemias who were exposed to tert-butyl hydroperoxide [26]. Deferiprone reduced brain iron accumulation in patients with pantothenate kinase-associated neurodegeneration, however this treatment did not induce clinical improvement in these patients [27].…”

Partial Splenectomy in the treatment of an adult with β thalassemia intermedia: A case report

“…A direct assessment of liver iron concentration is recommended in the follow-up of these patients, either by biopsy or by a non-invasive method such as T2 MRI. [2] , [3] , [6] .…”