Original article

Blood Transfusion - 3 2021 (May-June)

Changing patterns of thalassaemia in Italy: a WebThal perspective

Authors

Key words: thalassaemia, epidemiology, transfusion, iron overload, medical records
Publication Date: 2020-11-03

Abstract

Background - Migration has impacted the spread of thalassaemia which is gradually becoming a global health problem. Italy, with an approximate estimation of 7,000 patients, does not have an accurate national record for haemoglobinopathies. This cross-sectional evaluation includes data for approximately 50% of beta-thalassaemia patients in Italy to provide an overview of the burden of thalassaemia syndromes.
Materials and methods - The analysis included data on epidemiology, transfusions and clinical parameters from 3,986 thalassaemia patients treated at 36 centres in Italy who were alive on 31st December 2017. The study used WebThal, a computerised clinical record that is completely free-of-charge and that does not have any mandatory fields to be filled.
Results - For patients with thalassaemia major, 68% were aged ≥35 years and 11% were aged ≤18 years. Patients with thalassaemia intermedia were slightly older. Transfusion data, reported in a subgroup of 1,162 patients, showed 9% had pre-transfusion haemoglobin <9 g/dL, 63% had levels between ≥9 and <10 g/dL, and 28% had levels ≥10 g/dL. These 1,162 patients underwent 22,272 transfusion days during 2017, with a mean of 19 transfusion days/year/patient (range 1-54 days). Severity of iron overload was reported in 756 patients; many had moderate or mild liver iron load (74% had liver iron <7.5 mg/g dry weight). In the same cohort, 85% of patients had no signs of cardiac iron load (MRT2* >20 ms), and only 3% showed signs of high-risk heart condition (T2* <10 ms). Most patients had normal alanine amino transferase levels due to treatment with the new anti-hepatitis C virus (HCV) drugs.
Discussion - This study provides an overview of the current health status of patients with thalassaemia in Italy. Moreover, these data support the need for a national comprehensive thalassaemia registry.

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Authors

Filomena Longo - Reference Centre for Haemoglobinopathies, Department of Clinical and Biological Sciences, University of Turin, Orbassano, Italy

Paola Corrieri - Reference Centre for Haemoglobinopathies, Department of Clinical and Biological Sciences, University of Turin, Orbassano, Italy

Raffaella Origa - Thalassaemia Unit, "Antonio Cao" Paediatric-Hospital, AO Brotzu, Cagliari, Italy

Susanna Barella - Thalassaemia Unit, "Antonio Cao" Paediatric-Hospital, AO Brotzu, Cagliari, Italy

Paola M.G. Sanna - Immunohaematology and Transfusion Medicine Service, AOU Sassari, Sassari, Italy

Pier P. Bitti - Immunohaematology and Transfusion Medicine Service, ATS ASSL Nuoro, Nuoro, Italy

Angelo Zuccarelli - Immunohaematology and Transfusion Medicine Service, ATS ASSL Carbonia, Carbonia, Italy

Francesca V. Commendatore - Thalassaemia Unit, Lentini ASP 8 Siracusa, Siracusa, Italy

Angelantonio Vitucci - Haematology with Transplant Unit, Az. Universitario-Ospedaliera Consorziale Policlinico, Bari, Italy

Antonella Quarta - Microcythemia Center, Haematology with Transplant Unit, “A. Perrino” Hospital, Brindisi, Italy

Roberto Lisi - Thalassaemia Unit, ARNAS "Garibaldi", Catania, Italy

Maria D. Cappellini - Ca' Granda Foundation IRCCS, Dept. of Clinical Science and Community, University of Milan, Milan, Italy

Francesco Massei - Paediatric-Oncohaematology Unit, “Santa Chiara” Hospital, Pisa, Italy

Gian L. Forni - Center for Microcythemia, Congenital Anaemia and Iron Dysmetabolism, European Reference Networks for Rare Diseases ERN-EuroBloodNet, “Galliera” Hospital, Genoa, Italy

Antonio Piga - Reference Centre for Haemoglobinopathies, Department of Clinical and Biological Sciences, University of Turin, Orbassano, Italy

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