Your browser doesn't support javascript.
loading
The effect of desferrioxamine chelation versus no therapy in patients with non transfusion-dependent thalassaemia: a multicenter prospective comparison from the MIOT network.
Ricchi, Paolo; Meloni, Antonella; Pistoia, Laura; Spasiano, Anna; Spiga, Alessandra; Allò, Massimo; Gamberini, Maria Rita; Lisi, Roberto; Campisi, Saveria; Peluso, Angelo; Missere, Massimiliano; Renne, Stefania; Mangione, Maurizio; Positano, Vincenzo; Pepe, Alessia.
Afiliação
  • Ricchi P; Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Naples, Italy.
  • Meloni A; Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
  • Pistoia L; Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
  • Spasiano A; Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Naples, Italy.
  • Spiga A; U.O.C. Clinica Pediatrica Trasfusionale e Malattie Rare, Presidio Ospedaliero Pediatrico Microcitemico A. CAO, Cagliari, Italy.
  • Allò M; Servizio Microcitemia, Presidio Ospedaliero ASL 5, Crotone, Italy.
  • Gamberini MR; Dipartimento della Riproduzione e dell'Accrescimento Day Hospital della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria Arcispedale "S. Anna", Ferrara, Italy.
  • Lisi R; Unità Operativa Dipartimentale Talassemia, Presidio Ospedaliero Garibaldi-Centro-ARNAS Garibaldi, Catania, Italy.
  • Campisi S; Unità Operativa Semplice Talassemia, Presidio Ospedaliero Umberto I, Siracusa, Italy.
  • Peluso A; Struttura Semplice di Microcitemia, Ospedale SS. Annunziata ASL Taranto, Taranto, Italy.
  • Missere M; Dipartimento di Immagini, Fondazione di Ricerca e Cura "Giovanni Paolo II", Campobasso, Italy.
  • Renne S; Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero "Giovanni Paolo II", Lamezia Terme, Italy.
  • Mangione M; U.O.S. Sistemi informativi (UOSI), Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
  • Positano V; Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
  • Pepe A; Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy. alessia.pepe@ftgm.it.
Ann Hematol ; 97(10): 1925-1932, 2018 Oct.
Article em En | MEDLINE | ID: mdl-29926157
We prospectively assessed by magnetic resonance imaging (MRI) the advantages of desferrioxamine (DFO) with respect to the absence of chelation therapy in non transfusion-dependent thalassaemia (NTDT) patients. We considered 18 patients non-chelated and 33 patients who received DFO alone between the two MRI scans. Iron overload was assessed by the T2* technique. Biventricular function parameters were quantified by cine sequences. No patient treated with DFO had cardiac iron. At baseline, only one non-chelated patient showed a pathological heart T2* value (< 20 ms) and he recovered at the follow-up. The percentage of patients who maintained a normal heart T2* value was 100% in both groups. A significant increase in the right ventricular ejection fraction was detected in DFO patients (3.48 ± 7.22%; P = 0.024). The changes in cardiac T2* values and in the biventricular function were comparable between the two groups. In patients with hepatic iron at baseline (MRI liver iron concentration (LIC) ≥ 3 mg/g/dw), the reduction in MRI LIC values was significant only in the DFO group (- 2.20 ± 4.84 mg/g/dw; P = 0.050). The decrease in MRI LIC was comparable between the groups. In conclusion, in NTDT patients, DFO therapy showed no advantage in terms of cardiac iron but its administration allowed an improvement in right ventricular function. Moreover, DFO reduced hepatic iron in patients with significant iron burden at baseline.
Assuntos
Palavras-chave

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Talassemia / Terapia por Quelação / Quelantes de Ferro / Sobrecarga de Ferro / Desferroxamina Tipo de estudo: Clinical_trials / Observational_studies / Prognostic_studies Limite: Adolescent / Adult / Aged / Child / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2018 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Talassemia / Terapia por Quelação / Quelantes de Ferro / Sobrecarga de Ferro / Desferroxamina Tipo de estudo: Clinical_trials / Observational_studies / Prognostic_studies Limite: Adolescent / Adult / Aged / Child / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2018 Tipo de documento: Article