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Annals of Hematology

, Volume 97, Issue 10, pp 1925–1932 | Cite as

The effect of desferrioxamine chelation versus no therapy in patients with non transfusion-dependent thalassaemia: a multicenter prospective comparison from the MIOT network

  • Paolo Ricchi
  • Antonella Meloni
  • Laura Pistoia
  • Anna Spasiano
  • Alessandra Spiga
  • Massimo Allò
  • Maria Rita Gamberini
  • Roberto Lisi
  • Saveria Campisi
  • Angelo Peluso
  • Massimiliano Missere
  • Stefania Renne
  • Maurizio Mangione
  • Vincenzo Positano
  • Alessia Pepe
Original Article
  • 95 Downloads

Abstract

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.

Keywords

Desferrioxamine Non transfusion-dependent thalassaemia Magnetic resonance imaging Iron overload 

Notes

Acknowledgements

We would like to thank all the colleagues involved in the MIOT project (https://miot.ftgm.it/).

We thank Claudia Santarlasci for her skillful secretarial work. We finally thank all patients for their cooperation.

Funding

The MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A. and ApoPharma Inc.).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Paolo Ricchi
    • 1
  • Antonella Meloni
    • 2
  • Laura Pistoia
    • 2
  • Anna Spasiano
    • 1
  • Alessandra Spiga
    • 3
  • Massimo Allò
    • 4
  • Maria Rita Gamberini
    • 5
  • Roberto Lisi
    • 6
  • Saveria Campisi
    • 7
  • Angelo Peluso
    • 8
  • Massimiliano Missere
    • 9
  • Stefania Renne
    • 10
  • Maurizio Mangione
    • 11
  • Vincenzo Positano
    • 2
  • Alessia Pepe
    • 2
  1. 1.Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo RossoAzienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”NaplesItaly
  2. 2.Magnetic Resonance Imaging UnitFondazione G. Monasterio CNR-Regione ToscanaPisaItaly
  3. 3.U.O.C. Clinica Pediatrica Trasfusionale e Malattie RarePresidio Ospedaliero Pediatrico Microcitemico A. CAOCagliariItaly
  4. 4.Servizio MicrocitemiaPresidio Ospedaliero ASL 5CrotoneItaly
  5. 5.Dipartimento della Riproduzione e dell’Accrescimento Day Hospital della Talassemia e delle EmoglobinopatieAzienda Ospedaliero-Universitaria Arcispedale “S. Anna”FerraraItaly
  6. 6.Unità Operativa Dipartimentale TalassemiaPresidio Ospedaliero Garibaldi-Centro-ARNAS GaribaldiCataniaItaly
  7. 7.Unità Operativa Semplice TalassemiaPresidio Ospedaliero Umberto ISiracusaItaly
  8. 8.Struttura Semplice di MicrocitemiaOspedale SS. Annunziata ASL TarantoTarantoItaly
  9. 9.Dipartimento di ImmaginiFondazione di Ricerca e Cura “Giovanni Paolo II”CampobassoItaly
  10. 10.Struttura Complessa di Cardioradiologia-UTICPresidio Ospedaliero “Giovanni Paolo II”Lamezia TermeItaly
  11. 11.U.O.S. Sistemi informativi (UOSI)Fondazione G. Monasterio CNR-Regione ToscanaPisaItaly

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