ABSTRACT
Iron overload is a serious and potentially fatal condition that results from multiple blood transfusions required over a long period of time to treat certain types of anemias such as, that caused by β-thalassemia, sickle cell disease and myelodysplastic syndrome. Deferoxamine, which has been used since four decades as an iron chelator has limited efficacy due to its demanding therapeutic regimen, leading to poor compliance. Deferasirox, once daily oral iron chelator provides an effective alternative to Deferoxamine in the treatment of transfusional hemosiderosis. In this review, the role of Deferasirox as an ideal iron chelator has been discussed. Pubmed searches on Deferasirox were carried out for the same. Several studies demonstrated the safety and efficacy of Deferasirox in reducing iron burden in iron-overloaded patients with β-thalassemia, sickle cell anemia and myelodysplastic anemia. Thus, convenient, effective and tolerable chelation therapy with oral Deferasirox is likely to be a significant development in the treatment of transfusional iron overload, due to its ability to provide constant chelation coverage and the potential to improve compliance.
Subject(s)
Benzoates/chemistry , Benzoates/therapeutic use , Cardiovascular Diseases/epidemiology , Chelating Agents/chemistry , Chelating Agents/therapeutic use , Expert Testimony , Hemosiderosis/drug therapy , Hemosiderosis/epidemiology , Hemosiderosis/metabolism , Humans , Iron/metabolism , Liver/metabolism , Thalassemia/epidemiology , Thalassemia/metabolism , Triazoles/chemistry , Triazoles/therapeutic useABSTRACT
Oxygen-derived free radical damage is associated with the molecular toxicity of hemoglobin. Especially in thalassemia syndromes, this toxicity has a relationship with "free" alpha globin concentrations. This study of beta thalassemia trait blood samples from 39 individuals shows that the evaluation of methemoglobin is a sensitive method of indicating molecular toxicity and the superoxide dismutase concentration revealing the intensity of oxidative stress of this process.
Subject(s)
Humans , beta-Thalassemia , Clinical Laboratory Techniques , Erythrocytes/metabolism , Thalassemia/metabolismABSTRACT
Radicais livres derivados de espécies ativadas de oxigênio estao associados a lesoes citológicas, que diminuem a sobrevida dos eritrócitos, especialmente quando contêm defeitos congênitos, como sao os casos da anemia falciforme e da talassemia maior. As atividades antioxidantes dos eritrócitos dependem de três tipos de enzimas: superóxido dismutase, catalase e glutatiao peroxidase. Entretanto, as atividades antioxidantes podem ser ineficazes em situaçoes em que ocorrem o comprometimento dos hidroxiperóxidos da membrana eritrocitária, devido às células falciformes irreversíveis e à ligaçao de hemicromos à membrana dos eritrócitos talassêmicos.
Subject(s)
Humans , Anemia, Sickle Cell/metabolism , Erythrocytes/metabolism , Free Radicals , Thalassemia/metabolism , Anemia, Sickle Cell/enzymology , Erythrocytes/enzymology , Thalassemia/enzymologyABSTRACT
The mean +/- S.D. of LAP score in neutrophil of the normal control, non splenectomised patients and splenectomised patients were 116 +/- 78, 94 +/- 48 and 37 +/- 23 Kaplow units, respectively. The significant reduction in the leukocyte alkaline phosphatase level (P less than 0.05) was found in beta thalassemia/HbE patients. The degree of reduction was greater in the splenectomised patients (P less than 0.05). The decreased LAP levels in PMN was persistent. This was in contrast to the LAP in PMN of other patients where as LAP returned to normal after splenectomy. The relevance to the increased susceptibility to infection and low LAP in beta thalassemia/HbE should be further investigated.