Transfusional approach in multi-ethnic Sickle Cell patients: real-world practice data from a Multicenter survey in Italy
File(s)
Published version
Author(s)
Type
Journal Article
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.
Date Issued
2022-03-15
Date Acceptance
2022-02-14
Citation
Frontiers in Medicine, 2022, 9
ISSN
2296-858X
Publisher
Frontiers Media
Journal / Book Title
Frontiers in Medicine
Volume
9
Copyright Statement
Copyright © 2022 Graziadei, De Franceschi, Sainati, Venturelli, Masera, Bonomo,
Vassanelli, Casale, Lodi, Voi, Rigano, Pinto, Quota, Notarangelo, Russo, Allò, Rosso,
D’Ascola, Facchini, Macchi, Arcioni, Bonetti, Rossi, Sau, Campisi, Colarusso, Giona,
Lisi, Giordano, Boscarol, Filosa, Marktel, Maroni, Murgia, Origa, Longo, Bortolotti,
Colombatti, Di Maggio, Mariani, Piperno, Corti, Fidone, Palazzi, Badalamenti,
Gianesin, Piel and Forni. This is an open-access article distributed under the terms
of the Creative Commons Attribution License (CC BY). The use, distribution or
reproduction in other forums is permitted, provided the original author(s) and the
copyright owner(s) are credited and that the original publication in this journal
is cited, in accordance with accepted academic practice. No use, distribution or
reproduction is permitted which does not comply with these terms.
Vassanelli, Casale, Lodi, Voi, Rigano, Pinto, Quota, Notarangelo, Russo, Allò, Rosso,
D’Ascola, Facchini, Macchi, Arcioni, Bonetti, Rossi, Sau, Campisi, Colarusso, Giona,
Lisi, Giordano, Boscarol, Filosa, Marktel, Maroni, Murgia, Origa, Longo, Bortolotti,
Colombatti, Di Maggio, Mariani, Piperno, Corti, Fidone, Palazzi, Badalamenti,
Gianesin, Piel and Forni. This is an open-access article distributed under the terms
of the Creative Commons Attribution License (CC BY). The use, distribution or
reproduction in other forums is permitted, provided the original author(s) and the
copyright owner(s) are credited and that the original publication in this journal
is cited, in accordance with accepted academic practice. No use, distribution or
reproduction is permitted which does not comply with these terms.
License URL
Identifier
https://www.frontiersin.org/articles/10.3389/fmed.2022.832154/full
Publication Status
Published online
Date Publish Online
2022-03-16