Mediterranean Anemia: the first Campania center of the Miot national network is activated at Cardarelli

Per i pazienti talassemici la possibilità di completare il Percorso Diagnostico Terapeutico Assistenziale  con prestazioni di altissima qualità. Si annulla la necessità di spostarsi fuori regione.  Anna Iervolino: «Una risposta concreta ai nostri pazienti che si traduce anche in un risparmio per la Regione Campania di 100.000 euro l’anno».Un risparmio per le casse pubbliche di 100.000 euro l’anno, ma soprattutto la fine della migrazione sanitaria per i pazienti talassemici troppo spesso costretti ad andare fuori regione – in strutture specializzate – per essere inseriti in programmi di valutazione dei depositi di ferro negli organi. Disagi oggi cancellati grazie al lavoro del primo centro di Risonanza Magnetica in Campania della rete MIOT (Myocardial Iron Overload in Thalassemia) per la diagnosi e la cura dei soggetti affetti da Anemia Mediterranea. Il centro fa parte della struttura Complessa di Radiologia Generale e di PS (diretta  da Luigia Romano) del Cardarelli. L’UOSD Malattie rare del globulo rosso (diretta da Aldo Filosa) segue 200 pazienti talassemici dipendenti da trasfusioni continue, 140 pazienti con talassemia intermedia e 40 pazienti con drepanocitosi o microdrepanocitosi.

"The commitment of Cardarelli and the professionals who animate this company is maximum in all fields", says the Extraordinary Commissioner Anna Iervolino. "The opening of the MIOT center at Cardarelli was supported thanks to a regional financing (plan objectives 2018-2019) that made it possible to cover the costs of the validation of the center and periodic quality controls, as well as the costs for the training of the Team of Radiologists and Radiology Technicians. I am proud to remember that Cardarelli - concludes Iervolino - is a point of reference for many patients affected by Mediterranean Anemia, patients who need continuous blood transfusions to live. To these patients, in line with the firm intention of President Vincenzo De Luca to create a health system that is increasingly dedicated to excellence, we offer a true and complete response. A response that translates into quality of life for patients and their families and into savings".

Transfusions required by those suffering from Mediterranean Anemia have in fact as a side effect the accumulation of iron in the heart and in other organs such as the liver and pancreas with consequent complications such as heart problems (the main cause of death in adults affected by this pathology). Until the early 2000s the quantity of iron in the heart could not be quantified and therefore specific drugs for iron chelation were administered on the basis of indirect parameters such as serum ferritin, which has now been shown not to correlate with the accumulation of iron in the heart.

THE MIOT HEALTH SCIENTIFIC NETWORK

The network, which Cardarelli has joined since its inception (2006), has developed a diagnostic technique (T2* multislice multiecho) – shared by the centers belonging to the network – which, through Magnetic Resonance Imaging, is able to quantify in a non-invasive way the accumulation of cardiac iron (in the septum and in 15 other cardiac segments), hepatic and pancreatic iron with the direct consequence of being able to treat patients in a personalized way through iron chelating drugs.

Thanks to these methods and the marketing of new oral drugs to remove iron from the body (iron chelators), today the life expectancy of these patients has radically changed as demonstrated by scientific evidence recently published in the prestigious journal European Heart Journal Cardiovascular Imaging in 2018 (Pepe A et al), thanks to the cooperative effort of the MIOT network. «Patients with thalassemia, on average, need a magnetic resonance examination per year for the management of iron-chelating therapy – explains Alessia Pepe, scientific director and coordinator of the MIOT project at national level-. From the data of the MIOT study, it appears essential to perform MRI scans in close proximity for serious cardiac accumulations (every 3-6 months) resulting in much more frequent movement for the most compromised patients and also the possibility of evaluating iron accumulation early in pediatric age. The inconvenience for patients in Campania, an area with a high prevalence of thalassemia, was in accessing the specific Magnetic Resonance examination to evaluate the accumulation of cardiac, hepatic and pancreatic iron. An inconvenience that will be improved thanks to the new center of the Cardarelli in Naples. To date, in fact, within the MIOT network, a total of more than 9.000 examinations have been performed with 2.700 patients enrolled (of which 1.400 women and 200 pediatric age) who in many cases have significantly reduced the kilometers to travel to access treatment thanks to the presence of the network centers in the area.

Luigia Romano, scientific clinical manager for the radiological part of the MIOT project in the radiology department of the Cardarelli Hospital in Naples: «The center is equipped with a high-field GE Magnetic Resonance device (1.5 T) with a dedicated cardio-coil and software that can guarantee the availability of places for at least 18 patients per month». The Cardarelli center in Naples joins the current 9 Magnetic Resonance centers in Italy that make up the MIOT network led by the Fondazione Toscana Gabriele Monasterio CNR Regione Toscana in Pisa: Ancona, Campobasso, Catania, Ferrara, Lamezia Terme, Palermo Policlinico and Palermo Ospedale Civico, Pisa, Rome and Taranto. These centers guarantee the availability of more than 1.000 exams per year.

The network, which can be used by approximately 6.000 Italians affected by Mediterranean Anemia, includes 65 thalassemia centers. In Naples, Aldo Filosa, head of the UOSD Rare Red Blood Cell Diseases, is the clinical-scientific director for the hematological part of the MIOT project, which benefits from the collaboration of Paolo Ricchi and Anna Spasiano. These centers have decided to share their clinical-instrumental evaluations in a centralized web database, which is now recognized as one of the largest and most structured worldwide for thalassemia. Thanks to this database, over the last 13 years, 60 scientific works have been produced that have seen editorial light in prestigious international journals and in which the UOSD Rare Red Blood Cell Diseases group of the Cardarelli has actively participated as Authors and/or co-authors in 50% of the publications.

The entire operation took place under the ethical control of the Giambrone Italian Foundation for the healing of Thalassemia, a non-profit organization of patients' associations, chaired by Angela Iacono, who worked hard to activate the new MIOT center at Cardarelli.

The technique:

The Complex Unit of Specialized MRI and Neuroradiology of the Fondazione Toscana Gabriele Monasterio CNR Regione Toscana first developed the “multislice multiecho” technique that allows non-invasive quantification of cardiac iron accumulation throughout the left ventricle with a segmental approach that is more sensitive in identifying early accumulation states at cardiac level. The same Foundation validated a multislice multiecho approach for the quantification of pancreatic iron accumulation, considering that diabetes mellitus in the thalassemic population is an emerging cause of morbidity, and then verified the transferability of the approach to the Magnetic Resonance centers of the network, through a rigorous validation process on healthy volunteers and patients and through periodic quality control of the activated centers.