Research Paper
Assessment and management of iron overload in β-thalassaemia major patients during the 21st century: a real-life experience from the Italian Webthal project
Antonio Piga,
Filomena Longo,
Khaled M. Musallam,
Maria Domenica Cappellini,
Gian Luca Forni,
Giovanni Quarta,
Francesco Chiavilli,
Francesca Commendatore,
Sergio Mulas,
Vincenzo Caruso,
Renzo Galanello,
Corresponding Author
Antonio Piga
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
Correspondence: Antonio Piga, Department of Clinical and Biological Sciences, University of Turin, 10 Regione Gonzole, Orbassano, Turin 10043, Italy.
E-mail: [email protected]
Search for more papers by this authorFilomena Longo
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
Search for more papers by this authorKhaled M. Musallam
Department of Medicine and Medical Specialties, IRCCS Ca’ Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy
Search for more papers by this authorMaria Domenica Cappellini
Department of Medicine and Medical Specialties, IRCCS Ca’ Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy
Search for more papers by this authorGian Luca Forni
Centro della Microcitemia e Anemie Congenite, Ospedale Galliera, Genoa, Italy
Search for more papers by this authorGiovanni Quarta
Haematology Unit, “Di Summa” Hospital, Brindisi, Italy
Search for more papers by this authorFrancesco Chiavilli
Servizio di Immunoematologia e Trasfusione, S.I.T. ULSS 18, Rovigo, Italy
Search for more papers by this authorFrancesca Commendatore
U.O.S. di Talassemia, ASL. 8 Siracusa, Lentini, Italy
Search for more papers by this authorSergio Mulas
Centro Trasfusionale, Ospedale Civile di Alghero, Alghero, Italy
Search for more papers by this authorVincenzo Caruso
Unita' Operativa Dipartimentale Talassemia, P.O. S. Luigi-Curro, ARNAS Garibaldi, Catania, Italy
Search for more papers by this authorRenzo Galanello
Dipartimento Scienze Biomediche e Biotecnologie, Università di Cagliari, Ospedale Regionale Microcitemie, Cagliari, Italy
Search for more papers by this authorAntonio Piga,
Filomena Longo,
Khaled M. Musallam,
Maria Domenica Cappellini,
Gian Luca Forni,
Giovanni Quarta,
Francesco Chiavilli,
Francesca Commendatore,
Sergio Mulas,
Vincenzo Caruso,
Renzo Galanello,
Corresponding Author
Antonio Piga
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
Correspondence: Antonio Piga, Department of Clinical and Biological Sciences, University of Turin, 10 Regione Gonzole, Orbassano, Turin 10043, Italy.
E-mail: [email protected]
Search for more papers by this authorFilomena Longo
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
Search for more papers by this authorKhaled M. Musallam
Department of Medicine and Medical Specialties, IRCCS Ca’ Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy
Search for more papers by this authorMaria Domenica Cappellini
Department of Medicine and Medical Specialties, IRCCS Ca’ Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy
Search for more papers by this authorGian Luca Forni
Centro della Microcitemia e Anemie Congenite, Ospedale Galliera, Genoa, Italy
Search for more papers by this authorGiovanni Quarta
Haematology Unit, “Di Summa” Hospital, Brindisi, Italy
Search for more papers by this authorFrancesco Chiavilli
Servizio di Immunoematologia e Trasfusione, S.I.T. ULSS 18, Rovigo, Italy
Search for more papers by this authorFrancesca Commendatore
U.O.S. di Talassemia, ASL. 8 Siracusa, Lentini, Italy
Search for more papers by this authorSergio Mulas
Centro Trasfusionale, Ospedale Civile di Alghero, Alghero, Italy
Search for more papers by this authorVincenzo Caruso
Unita' Operativa Dipartimentale Talassemia, P.O. S. Luigi-Curro, ARNAS Garibaldi, Catania, Italy
Search for more papers by this authorRenzo Galanello
Dipartimento Scienze Biomediche e Biotecnologie, Università di Cagliari, Ospedale Regionale Microcitemie, Cagliari, Italy
Search for more papers by this authorSummary
We conducted a cross-sectional study on 924 β-thalassaemia major patients (mean age 30·1 years) treated at nine Italian centres using the webthal software, to evaluate real-life application of iron overload assessment and management standards. Serum ferritin <2500 ng/ml was a risk factor for never having liver iron concentration (LIC) measurement, while absence of cardiac disease and siderosis were risk factors for a delay in LIC measurement >2 years. Patients who never had a cardiac MRI (CMR) T2* measurement were <18 years, had iron intake ≤0·4 mg/kg per day, or a serum ferritin <2500 ng/ml. A history of normal CMR T2* was the main risk factor for a delay in subsequent assessment of >2 years. Deferoxamine (22·8%) was more commonly used in patients with Hepatitis C Virus or high serum creatinine. Deferiprone (20·6%) was less commonly prescribed in patients with elevated alanine aminotransferase; while a deferoxamine + deferiprone combination (17·9%) was more commonly used in patients with serum ferritin >2500 ng/ml or CMR T2* <20 ms. Deferasirox (38·3%) was more commonly prescribed in patients <18 years, but less commonly used in those with heart disease or high iron intake. These observations largely echoed guidelines at the time, although some practices are expected to change in light of evolving evidence.
Supporting Information
| Filename | Description |
|---|---|
| bjh12340-sup-0001-Supplement1.pdfapplication/PDF, 41.7 KB | Fig S1. Distribution of patients among participating centers. |
| bjh12340-sup-0002-Supplement2.pdfapplication/PDF, 19.7 KB | Fig S2. Age distribution of patients. |
Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
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