Transfusional Approach in Multi-Ethnic Sickle Cell Patients: Real-World Practice Data From a Multicenter Survey in Italy
Year: 2022
Type: article
Study design: Observational Study
Abstract: Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder characterized by recurrent acute vaso-occlusive crises (VOCs and anemia). Gold standard treatments are hydroxycarbamide (HC) and/or different red blood cell (RBC) transfusion regimens to limit disease progression. Here, we report a retrospective study on 1,579 SCD patients (median age 23 years; 802 males/777 females), referring to 34 comprehensive Italian centers for hemoglobinopathies. Although we observed a similar proportion of Caucasian (47.9%) and African (48.7%) patients, Italian SCD patients clustered into two distinct overall groups: children of African descent and adults of Caucasian descent. We found a subset of SCD patients requiring more intensive therapy with a combination of HC plus chronic transfusion regimen, due to partial failure of HC treatment alone in preventing or reducing sickle cell-related acute manifestations. Notably, we observed a higher use of acute transfusion approaches for SCD patients of African descent when compared to Caucasian subjects. This might be related to (i) age of starting HC treatment; (ii) patients' low social status; (iii) patients' limited access to family practitioners; or (iv) discrimination. In our cohort, alloimmunization was documented in 135 patients (8.5%) and was more common in Caucasians (10.3%) than in Africans (6.6%). Alloimmunization was similar in male and female and more frequent in adults than in children. Our study reinforces the importance of donor-recipient exact matching for ABO, Rhesus, and Kell antigen systems for RBC compatibility as a winning strategy to avoid or limit alloimmunization events that negatively impact the clinical management of SCD-related severe complications. Clinical Trial Registration: ClinicalTrials.gov, identifier: NCT03397017.
Source: Frontiers in Medicine
Authors: Giovanna Graziadei, Lucia De Franceschi, Laura Sainati, Donatella Venturelli, Nicoletta Masera, Piero Bonomo, Aurora Vassanelli, Maddalena Casale, Gianluca Lodi, Vincenzo Voi, Paolo Rigano, Valeria Maria Pinto, Alessandra Quota, Lucia D. Notarangelo, Giovanna Russo, Massimo Allò, Rosamaria Rosso, Domenico D'Ascola, Elena Facchini, Silvia Macchi, Francesco Arcioni, Federico Bonetti, Enza Rossi, Antonella Sau, Saveria Campisi, Gloria Colarusso, Fiorina Giona, Roberto Lisi, Paola Giordano, Gianluca Boscarol, Aldo Filosa, Sarah Marktel, Paola Maroni, Mauro Murgia, Raffaella Origa, Filomena Longo, Marta Bortolotti, Raffaella Colombatti, Rosario Di Maggio, Raffaella Mariani, Alberto Piperno, Paola Corti, Carmelo Fidone, Giovanni Palazzi, Luca Badalamenti, Barbara Gianesin, Frédéric B. Piel, Gian Luca Forni
Institutions: Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Verona, Azienda Ospedaliera Universitaria Integrata Verona, Azienda Ospedale - Università Padova, Azienda Ospedaliero-Universitaria di Modena, Azienda Ospedaliera San Gerardo, University of Milano-Bicocca, University of Campania "Luigi Vanvitelli", Arcispedale Sant'Anna, Ospedale San Luigi Gonzaga, University of Turin, Ospedale Vincenzo Cervello, Ente Ospedaliero Ospedali Galliera, Associazione Italiana Ematologia Oncologia Pediatrica, University of Catania, Azienda Ospedaliero Universitaria Policlinico "G.Rodolico - San Marco", Azienda ospedaliera "Bianchi-Melacrino-Morelli", IRCCS Azienda Ospedliero-Universitaria di Bologna Policlinico di Sant'Orsola, Ospedale "Santa Maria delle Croci" di Ravenna, Policlinico San Matteo Fondazione, Azienda Ospedaliera di Cosenza, Ospedale Annunziata di Cosenza, Ospedale di Prato Santo Stefano, Sapienza University of Rome, Ospedale Garibaldi, University of Bari Aldo Moro, Ospedale di Bolzano, Ospedale Antonio Cardarelli, IRCCS Ospedale San Raffaele, Aziende Socio Sanitarie Territoriale dei Sette Laghi, University of Cagliari, Ospedale Microcitemico, University of Milan, University of Palermo, Imperial College London
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,950
OpenAlex ID: W4220924684