Annals of Hematology

, Volume 91, Issue 9, pp 1443–1449

Cardiac iron removal and functional cardiac improvement by different iron chelation regimens in thalassemia major patients

  • Elena Cassinerio
  • Alberto Roghi
  • Patrizia Pedrotti
  • Francesca Brevi
  • Laura Zanaboni
  • Giovanna Graziadei
  • Paolo Pattoneri
  • Angela Milazzo
  • Maria Domenica Cappellini
Original Article

DOI: 10.1007/s00277-012-1480-8

Cite this article as:
Cassinerio, E., Roghi, A., Pedrotti, P. et al. Ann Hematol (2012) 91: 1443. doi:10.1007/s00277-012-1480-8

Abstract

Heart failure due to myocardial iron overload remains the leading cause of morbidity and mortality in adult thalassemia major (TM) patients. We evaluated the removal of cardiac iron and the changes of cardiac function by different iron chelation in TM patients by T2* cardiac magnetic resonance (CMR). Sixty-seven TM patients (27 males/40 females; mean age, 35 ± 6 years) on different chelation regimens underwent T2* CMR at baseline (t0), after 6–14 months (t1) and after 32 ± 7 months (t2). Patients were divided in four groups according to chelation treatment: group A (deferasirox), group B (deferoxamine), group C (combined treatment, deferoxamine plus deferiprone) and group D (deferiprone alone). Myocardial T2* at t0 was <10 ms in 8 patients, between 10 and 20 ms in 22 patients and ≥20 ms in 37 patients. Progressive changes in T2* were observed at t1 and t2. Ten patients (10/36, 27.8 %) in group A, three patients (3/15, 20 %) in group B and three patients (3/12, 25 %) in group C moved from an abnormal T2* to normal values. We observed an improvement of left ventricular ejection fraction and a reduction of end-systolic and end-diastolic left ventricular volumes only in patients in group A with baseline cardiac T2* between 10 and 20 ms. Rigorous compliance to any chelation therapy at proper doses significantly improve myocardial T2*. Treatment with deferasirox significantly improves left ventricular function. Combination therapy seems to ameliorate cardiac T2* in a shorter period of time in severe siderosis.

Keywords

Thalassemia major Myocardial siderosis Chelation therapy Cardiac magnetic resonance T2* Cardiac function 

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Elena Cassinerio
    • 1
  • Alberto Roghi
    • 2
  • Patrizia Pedrotti
    • 2
  • Francesca Brevi
    • 1
  • Laura Zanaboni
    • 1
  • Giovanna Graziadei
    • 1
  • Paolo Pattoneri
    • 1
  • Angela Milazzo
    • 2
  • Maria Domenica Cappellini
    • 1
    • 3
  1. 1.Hereditary Anemia Centre, Department of Internal Medicine, “Ca’ Granda” Foundation Ospedale Maggiore Policlinico IRCCSUniversity of MilanMilanItaly
  2. 2.CMR Unit, Department of CardiologyNiguarda Ca’ Granda HospitalMilanItaly
  3. 3.Hereditary Anemia Center -Pad. Granelli- Fondazione IRCCS Cà Granda Ospedale Maggiore PoliclinicoMilanItaly

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