Chelation therapy with desferrioxamine does not normalize ferritin level but attenuates oxidative damage and improves total antioxidant level in malaysian Chinese β-thalassaemia major patients / La terapia de quelación con deferoxamina no normaliza los niveles de ferritinaa pero atenúa el daño oxidativo y mejora el nivel antioxidante total en los pacientes sinomalayos que padecen de talasemia β

Beta-thalassaemia major causes severe anaemia and patients with it may be transfusion-dependent for life. Regular blood transfusions cause iron-overload that leads to oxidative damage which can hasten mortality. The objective of this research was to study the oxidant-antioxidant indices in β-thalassaemia major patients at the University of Malaya Medical Centre (UMMC) who were on desferrioxaminechelation or without chelation therapy. Blood was collected from 39 Chinese patients and 20 controls. Plasma and peripheral blood mononuclear cell lysates (PBMC) were extracted and biochemical tests to evaluate oxidative stress were performed. Oxidative stress was evident in these patients as advanced oxidized protein products (AOPP) and lipid hydroperoxides were elevated, whereas glutathione peroxidase activity and the ferric reducing antioxidant power (FRAP) were reduced. The catalase activity in the patients' PBMC was elevated, possibly as a compensatory mechanism for the reduced glutathione peroxidase activity in both red blood cells and PBMC. The lower FRAP and higher AOPP levels in the non-chelated patients compared with the chelated patients were indicative of a lower oxidative stress level in the chelated patients. The ferritin levels in the chelated and non-chelated patients were high and the mean levels of liver enzyme activities in the majority of patients were elevated regardless of chelation therapy. In conclusion, this study indicates that desferrioxamine chelation therapy does not normalize ferritin level but attenuates oxidative damage and improves total antioxidant level in Malaysian Chinese β-thalassaemia major patients.

La beta-talasemia mayor causa anemia severa, y los pacientes con este padecimiento pueden hacerse dependientes de las transfusiones de sangre por el resto de sus vidas. Las transfusiones regulares de sangre dan lugar a una sobrecarga de hierro que conduce al dano oxidativo, el cual a su vez puede acelerar la mortalidad. El objetivo de esta investigación fue estudiar las tasas de oxidantesantioxidantes en pacientes de beta-talasemia mayor en el Centro Médico de la Universidad de Malaya, tanto aquellos bajo tratamiento de quelación con deferoxamina, como aquellos sin terapia de quelación alguna. Se recogieron muestras de sangre de 39 pacientes chinos y 20 controles. Se extrajeron plasma y lisados de celulas mononucleares perifericas (CMSP), y se realizaron pruebas bioquimicas para evaluar el estrés oxidativo. El estrés oxidativo era evidente en estos pacientes en forma de productos avanzados de oxidación de proteinas (PAOP), y los hidroperoxidos de lipidos eran elevados, en tanto que la actividad de glutatión peroxidasa y el poder reductor ferrico/antioxidante (FRAP) era reducida. La actividad de la catalasa en los pacientes de CMSP era elevada, posiblemente como un mecanismo compensatorio frente a la actividad de glutatión peroxidasa reducida tanto en los globulos rojos como en las CMSP. Los niveles más bajos de FRAP y los más altos de PAOP en los pacientes no quelados en comparación con los pacientes quelados, indicaban un bajo nivel de estrés oxidativo en los pacientes quelados. Los niveles de ferritina tanto en los pacientes quelados como en los no quelados, eran altos, y los niveles promedio de actividades enzimaticas del higado fueron elevados en la mayoria de los pacientes, independientemente de la terapia de quelación. En conclusión, este estudio indica que la terapia de quelación con deferoxamina no normaliza el nivel de ferritina, pero en cambio atenua el daño oxidativo, y mejora el nivel antioxidante total en los pacientes sinomalayos afectados por la betatalasemia mayor.

Relationship between elevated liver enzyme with iron overload and viral hepatitis in thalassemia major patients in Northern Iran

To determine the relationship between elevated liver enzymes with iron overload and viral hepatitis in thalassemic patients. This descriptive cross-sectional study was carried out in the thalassemic ward of Tonekabon Hospital, Mazandaran, Northern Iran from 20 April to 20 September of 2006. Patients were studied with respect to age, liver enzymes, anti-hepatitis C virus [anti-HCV] antibody, and hepatitis B surface antigen [HBsAg], transferrin saturation [TSAT], and blood transfusion index [multiplication of frequency and units of transfusion]. Alanine aminotransferase [ALT] >/= 40 U/L was considered elevated. Sixty-five patients were evaluated [median age 19.51 +/- 8.9 years, range 4-54]. Eleven patients were anti-HCV positive [16.9%]. The mean serum ferritin was significantly higher in patients with ALT >/= 40 [2553.08 micro g/L versus 1783.7750 micro g/L] [p=0.012]. The mean ALT was significantly higher in patients with TSAT >/= 60% [41.26 U/L versus 28.82 U/L] [p=0.021]. The relationship between ALT >/= 40 and anti-HCV positivity was statistically significant. The mean ALT was 60.91 U/L in anti-HCV positive ptients and 39.29 U/L in the negative group [p=0.001]. The mean serum iron and transfusion index were significantly higher in anti-HCV positive versus negative patients [234.0 versus 195.4815; p=0.02], [1693.6 versus 1036.29, p=0.014]. Close association between elevated ALT with iron overload, transfusion index, age, and anti-HCV positivity in thalassemic patients of Tonekabon is recommended to re-evaluate transfusion and Desferal doses and therapies other than blood transfusion