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

Graziadei, G;De Franceschi, L;Sainati, L;Venturelli, D;Masera, N;Bonomo, P;Vassanelli, A;Casale, M;Lodi, G;Voi, V;Rigano, P;Pinto, VM;Quota, A;Notarangelo, LD;Russo, G;Allo, M;Rosso, R;D'Ascola, D;Facchini, E;Macchi, S;Arcioni, F;Bonetti, F;Rossi, E;Sau, A;Campisi, S;Colarusso, G;Giona, F;Lisi, R;Giordano, P;Boscarol, G;Filosa, A;Marktel, S;Maroni, P;Murgia, M;Origa, R;Longo, F;Bortolotti, M;Colombatti, R;Di Maggio, R;Mariani, R;Piperno, A;Corti, P;Fidone, C;Palazzi, G;Badalamenti, L;Gianesin, B;Piel, FB;Forni, GL

2022-01-01

---

Abstract

Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disordercharacterized by recurrent acute vaso-occlusive crises (VOCs and anemia). Goldstandard treatments are hydroxycarbamide (HC) and/or different red blood cell (RBC)transfusion regimens to limit disease progression. Here, we report a retrospective studyon 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 identifier: NCT03397017.