Original article

Vol. 22 No. 1 (2024): Blood Transfusion 1-2024 (January-February)

Longitudinal prospective comparison of pancreatic iron by magnetic resonance in thalassemia patients transfusion-dependent since early childhood treated with combination deferiprone-desferrioxamine vs deferiprone or deferasirox monotherapy

Authors

Key words: thalassemia, chelating agents, magnetic resonance imaging, iron overload, pancreas
Publication Date: 2023-02-24

Abstract

Background - In transfusion-dependent thalassemia patients who started regular transfusions in early childhood, we prospectively and longitudinally evaluated the efficacy on pancreatic iron of a combined deferiprone (DFP) + desferrioxamine (DFO) regimen versus either oral iron chelator as monotherapy over a follow-up of 18 months.
Materials and methods - We selected patients consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia network who received a combined regimen of DFO+DFP (N=28) or DFP (N=61) or deferasirox (DFX) (N=159) monotherapy between the two magnetic resonance imaging scans. Pancreatic iron overload was quantified by the T2* technique.
Results - At baseline no patient in the combined treatment group had a normal global pancreas T2* (≥26 ms). At follow-up the percentage of patients who maintained a normal pancreas T2* was comparable between the DFP and DFX groups (57.1 vs 70%; p=0.517).
Among the patients with pancreatic iron overload at baseline, global pancreatic T2* values were significantly lower in the combined DFO+DFP group than in the DFP or DFX groups. Since changes in global pancreas T2* values were negatively correlated with baseline pancreas T2* values, the percent changes in global pancreas T2* values, normalized for the baseline values, were considered. The percent changes in global pancreas T2* values were significantly higher in the combined DFO+DFP group than in either the DFP (p=0.036) or DFX (p=0.030) groups.
Discussion - In transfusion-dependent patients who started regular transfusions in early childhood, combined DFP+DFO was significantly more effective in reducing pancreatic iron than was either DFP or DFX.

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Authors

Paolo Ricchi U.O.S.D. Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Naples, Italy

Antonella Meloni Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy; U.O.C. Bioingegneria, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Laura Pistoia Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Maria R. Gamberini Unità Operativa di Day Hospital della Talassemia e delle Emoglobinopatie, Dipartimento della Riproduzione e dell’Accrescimento, Azienda Ospedaliero-Universitaria "S. Anna", Cona (FE), Italy

Liana Cuccia Unità Operativa Complessa Ematologia con Talassemia, ARNAS Civico “Benfratelli-Di Cristina”, Palermo, Italy

Massimo Allò Ematologia Microcitemia, Ospedale San Giovanni di Dio-ASP Crotone, Crotone, Italy

Maria C. Putti Dipartimento della Salute della Donna e del Bambino, Clinica di Emato-Oncologia Pediatrica, Azienda Ospedaliero-Università di Padova, Padua, Italy

Anna Spasiano U.O.S.D. Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Naples, Italy

Rosamaria Rosso Unità Operativa Talassemie ed Emoglobinopatie Azienda Ospedaliero-Universitaria Policlinico “Vittorio Emanuele”, Catania, Italy

Valerio Cecinati Struttura Semplice di Microcitemia, Ospedale “SS. Annunziata” ASL Taranto, Taranto, Italy

Riccardo Righi Diagnostica per Immagini e Radiologia Interventistica, Ospedale del Delta, Lagosanto, Italy

Stefania Renne Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero “Giovanni Paolo II”, Lamezia Terme (CZ), Italy

Giuseppe Peritore Unità Operativa Complessa di Radiologia, ARNAS Civico “Benfratelli-Di Cristina”, Palermo, Italy

Antonino Vallone Reparto di Radiologia, Azienda Ospedaliera "Garibaldi" Presidio Ospedaliero Nesima, Catania, Italy

Vincenzo Positano Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy; U.O.C. Bioingegneria, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Emilio Quaia Institute of Radiology, Department of Medicine, University of Padua, Padua, Italy

Filippo Cademartiri Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Alessia Pepe Institute of Radiology, Department of Medicine, University of Padua, Padua, Italy

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