Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Transfusional iron overload and chelation therapy with deferoxamine and deferiprone (L1)
Autore:
Kontoghiorghes, GJ; Pattichi, K; Hadjigavriel, M; Kolnagou, A;
Indirizzi:
Postgrad Res Inst Sci Technol Environm & Med, CY-3021 Limassol, Cyprus Postgrad Res Inst Sci Technol Environm & Med Limassol Cyprus CY-3021 rus
Titolo Testata:
TRANSFUSION SCIENCE
fascicolo: 3, volume: 23, anno: 2000,
pagine: 211 - 223
SICI:
0955-3886(200012)23:3<211:TIOACT>2.0.ZU;2-4
Fonte:
ISI
Lingua:
ENG
Soggetto:
LONG-TERM TREATMENT; THALASSEMIA MAJOR; 1,2-DIMETHYL-3-HYDROXYPYRID-4-ONE L1; LOADED PATIENTS; EFFICACY; TRIAL; DESFERRIOXAMINE; MOBILIZATION; TOXICITY; AGRANULOCYTOSIS;
Keywords:
oral chelators; iron overload; deferoxamine; L1; deferiprone; thalassaemia;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
79
Recensione:
Indirizzi per estratti:
Indirizzo: Kontoghiorghes, GJ Postgrad Res Inst Sci Technol Environm & Med, 3 Ammochostou St, CY-3021 Limassol, Cyprus Postgrad Res Inst Sci Technol Environm & Med 3 Ammochostou St Limassol Cyprus CY-3021
Citazione:
G.J. Kontoghiorghes et al., "Transfusional iron overload and chelation therapy with deferoxamine and deferiprone (L1)", TRANSFUS SC, 23(3), 2000, pp. 211-223

Abstract

Iron is essential for all living organisms. Under normal conditions there is no regulatory and rapid iron excretion in humans and body iron levels are mainly regulated from the absorption of iron from the gut. Regular blood transfusions in thalassaemia and other chronic refractory anaemias can result in excessive iron deposition in tissues and organs. This excess iron is toxic, resulting in tissue and organ damage and unless it is removed it canbe fatal to those chronically transfused. Iron removal in transfusional iron overload is achieved using chelation therapy with the chelating drugs deferoxamine (DF) and deferiprone (L1). Effective chelation therapy in chronically transfused patients can only be achieved if iron chelators can removesufficient amounts of iron, equivalent to those accumulated in the body from transfusions, maintaining body iron load at a non-toxic level. In order to maintain a negative iron balance, both chelating drugs have to be administered almost daily and at high doses. This form of administration also requires that a chelator has low toxicity, good compliance and low cost. DF has been a life-saving drug for thousands of patients in the last 40 years. It is mostly administered by subcutaneous infusion (40-60 mg/kg, 8-12 h, 5 days per week), is effective in iron removal and has low toxicity. However, less than 10% of the patients requiring iron chelation therapy worldwide are able to receive DF because of its high cost, low compliance and in some cases toxicity. In the last 10 years we have witnessed the emergence of oralchelation therapy, which could potentially change the prognosis of all transfusional iron-loaded patients. The only clinically available oral iron chelator is L1, which has so far been taken by over 6000 patients worldwide, in some cases daily for over 10 years, with very promising results. L1 was able to bring patients to a negative iron balance at doses of 50-120 mg/kg/day. It increases urinary iron excretion, decreases serum ferritin levels and reduces liver iron in the majority of chronically transfused iron-loadedpatients. Despite earlier concerns of possible increased risk of toxicity,all the toxic side effects of L1 are currently considered reversible, controllable and man ageable. These include agranulocytosis (0.6%), musculoskeletal and joint pains (15%), gastrointestinal complaints (6%) and zinc deficiency (1%). The incidence of these toxic side effects could in general be reduced by using lower doses of L1 or combination therapy with DF. Combination therapy could also benefit patients experiencing toxicity with DF and those not responding to either chelator alone. The overall efficacy and toxicity of L1 is comparable to that of DF in both animals and humans. Despite the steady progress in iron chelation therapy with DF and L1, further investigations are required for optimising their use in patients by selecting improved dose protocols, by minimising their toxicity and by identifying new applications in other diseases of iron imbalance. (C) 2000 Elsevier Science Ltd. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 10/04/20 alle ore 02:19:32