Frontiers in Medicine (Mar 2022)

Transfusional Approach in Multi-Ethnic Sickle Cell Patients: Real-World Practice Data From a Multicenter Survey in Italy

  • 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

DOI
https://doi.org/10.3389/fmed.2022.832154
Journal volume & issue
Vol. 9

Abstract

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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 RegistrationClinicalTrials.gov, identifier: NCT03397017.

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