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Hematology. 2016 Aug;21(7):415-24. doi: 10.1080/10245332.2015.1101971. Epub 2016 Feb 22.

The Italian multiregional thalassemia registry: Centers characteristics, services, and patients' population.

Author information

1
a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Via Abate Eustasio 30, 70010 Valenzano (BA) , Italy.
2
b Consorzio per Valutazioni Biologiche e Farmacologiche , Via L. Porta 14, 27100 Pavia , Italy.
3
c Fondazione Salvatore Maugeri , Via S. Maugeri, 4, 27100 Pavia , Italy.
4
d Fondazione G. Monasterio C.N.R.- Regione Toscana , Via Moruzzi 1, 56124 Pisa , Italy.
5
e Azienda Ospedaliera Padova Centro Leucemie Oncoematologia Pediatrica , Via N. Giustiniani 3, 35128 Padova , Italy.
6
f Azienda Ospedaliera Universitaria Consorziale Policlinico di Bari, Unità Operativa di Pediatria Generale e Specializtica "Federico Vecchio" , P.zza G. Cesare, 11, 70124 Bari , Italy.
7
g Azienda Ospedaliera Ospedali Riuniti Villa Sofia Cervello , Via Trabucco, 180, 90146 Palermo , Italy.
8
h Azienda Ospedaliera "A. Cardarelli" UOS Talassemia Pediatrica e Emoglobinopatie Pediatriche , Via A. Cardarelli, 9, 80131 Napoli , Italy.
9
i Fondazione "Leonardo Giambrone" per la Guarigione dalla Thalassemia , Via Savona, 56, 81030 Castelvolturno (CE) , Italy.

Abstract

OBJECTIVES:

The prognosis of beta-Thalassemia major and other congenital hemoglobinopathies has profoundly changed over the last decades. Moreover, only few countries in Europe provide dedicated services and the description of the measures for patients monitoring and treatment is overall very scarce. The HTA-Thal project is aimed to identify the services available in Italy and to collect epidemiological and clinical data on the thalassemic population (HTA-Thal Registry).

METHODS:

A map of the existing centers was created and two electronic questionnaires were completed with information on the services and patients.

RESULTS:

On 182 centers identified, 60 completed the two questionnaires. Centers resulted to be extremely heterogeneous in terms of size, age of patients in care, and services availability. The transition of pediatric patients to adult centers was not guaranteed. Thousand eight hundred and seventy-three beta-Thalassemia major patients (of which 259 pediatrics), regularly transfused, were registered. Deferasirox is the most used chelator as monotherapy (616 patients) and its use prevails in younger patients. A higher number of patients (847 patients) use Deferoxamine, either alone (448 patients) or in combination with DFP (399 patients), while 782 patients use Deferiprone alone (383 patients) or in combination (399 patients). 31.6 and 66.6% of centers were not equipped for specialized visits or local MRI, respectively. Centers with 30-80 patients show the high percentage of patients appropriately monitored when compared to smaller or bigger centers.

CONCLUSIONS:

This analysis confirms the importance of patients' registries for the collection of large datasets and the need for dedicated 'specialized centers' equipped to provide the best standard treatment to patients.

KEYWORDS:

Beta-Thalassemia; Chelation; Hemoglobinopathies centers; MRI; Registries; Transfusion

PMID:
26863102
DOI:
10.1080/10245332.2015.1101971
[Indexed for MEDLINE]

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