Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging.
BACKGROUND
Oral deferiprone was suggested to be more effective than subcutaneous desferrioxamine for removing heart iron. Oral once-daily chelator deferasirox has recently been made commercially available but its long-term efficacy on cardiac iron and function has not yet been established. Our study aimed to compare the effectiveness of deferasirox, deferiprone and desferrioxamine on myocardial and liver iron concentrations and bi-ventricular function in thalassemia major patients by means of quantitative magnetic resonance imaging.


METHODS
From the first 550 thalassemia subjects enrolled in the Myocardial Iron Overload in Thalassemia network, we retrospectively selected thalassemia major patients who had been receiving one chelator alone for longer than one year. We identified three groups of patients: 24 treated with deferasirox, 42 treated with deferiprone and 89 treated with desferrioxamine. Myocardial iron concentrations were measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. Liver iron concentrations were measured by T2* multiecho technique.


RESULTS
The global heart T2* value was significantly higher in the deferiprone (34 ± 11 ms) than in the deferasirox (21 ± 12 ms) and the desferrioxamine groups (27 ± 11 ms) (P = 0.0001). We found higher left ventricular ejection fractions in the deferiprone and the desferrioxamine versus the deferasirox group (P = 0.010). Liver iron concentration, measured as T2* signal, was significantly lower in the desferrioxamine versus the deferiprone and the deferasirox group (P = 0.004).


CONCLUSIONS
The cohort of patients treated with oral deferiprone showed less myocardial iron burden and better global systolic ventricular function compared to the patients treated with oral deferasirox or subcutaneous desferrioxamine.
Pepe
Alessia
A
Magnetic Resonance Imaging Unit, Fondazione G. Monasterio, C.N.R. - Regione Toscana, Pisa, Italy. alessia.pepe@ifc.cnr.it
Meloni
Antonella
A
Capra
Marcello
M
Cianciulli
Paolo
P
Prossomariti
Luciano
L
Malaventura
Cristina
C
Putti
Maria Caterina
MC
Lippi
Alma
A
Romeo
Maria Antonietta
MA
Bisconte
Maria Grazia
MG
Filosa
Aldo
A
Caruso
Vincenzo
V
Quarta
Antonella
A
Pitrolo
Lorella
L
Missere
Massimiliano
M
Midiri
Massimo
M
Rossi
Giuseppe
G
Positano
Vincenzo
V
Lombardi
Massimo
M
Maggio
Aurelio
A
D000293:Adolescent; D000328:Adult; D001565:Benzoates / Q000627:therapeutic use*; D002648:Child; D000077588:Deferasirox; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000139:chemically induced / Q000188:drug therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011728:Pyridones / Q000627:therapeutic use*; D012189:Retrospective Studies; D017262:Siderophores / Q000627:therapeutic use; D014230:Triazoles / Q000627:therapeutic use*; D016276:Ventricular Function / Q000187:drug effects*; D055815:Young Adult; D017086:beta-Thalassemia / Q000188:drug therapy*
D001565:Benzoates; D007502:Iron Chelating Agents; D011728:Pyridones; D017262:Siderophores; D014230:Triazoles; D000077543:Deferiprone; D007501:Iron; D003676:Deferoxamine; D000077588:Deferasirox