Abstract
β-thalassemia major (βTM) patients require frequent blood transfusions, with consequences that span from allogenic reactions to iron overload. To minimize these effects, βTM patients periodically receive leucodepleted packed red blood cells (P-RBCs) stored for maximum 14 days. The aim of this study was to compare two alternative routine procedures to prepare the optimal P-RBCs product, in order to identify differences in their content that may somehow affect patients’ health and quality of life (QoL). In method 1, blood was leucodepleted and then separated to obtain P-RBCs, while in method 2 blood was separated and leucodepleted after removal of plasma and buffycoat. Forty blood donors were enrolled in two independent centers; couples of phenotypically matched whole blood units were pooled, divided in two identical bags and processed in parallel following the two methods. Biochemical properties, electrolytes and metabolic composition were tested after 2, 7 and 14 days of storage. Units prepared with both methods were confirmed to have all the requirements necessary for βTM transfusion therapy. Nevertheless, RBCs count and Hb content were found to be higher in method-1, while P-RBCs obtained with method 2 contained less K+, iron and storage lesions markers. Based on these results, both methods should be tested in a clinical perspective study to determine a possible reduction of transfusion-related complications, improving the QoL of βTM patients, which often need transfusions for the entire lifespan.
Highlights
Introduction iationsThe defective synthesis of the β-globin chain of hemoglobin (Hb) is the cause of a group of hereditary blood diseases named β-thalassemias
40 blood donors, we investigated how the two procedures affect the features of packed red blood cells (P-red blood cells (RBCs)) destined to β-thalassemia major (βTM) patients, assessing hematologic parameters, hemolysis, major metabolites content, iron metabolism, electrolytes and cytokines concentrations
In P-RBCs prepared with method 1 we found a higher concentration of both Hb and iron, which in turn may result in the development of reactive oxygen species (ROS) with a higher rate
Summary
The defective synthesis of the β-globin chain of hemoglobin (Hb) is the cause of a group of hereditary blood diseases named β-thalassemias. In the case of β-thalassemia major (βTM) the absence of β-globin chains induces massive red blood cells (RBCs) hemolysis that leads to a severe pathological condition and eventually to death, if untreated, during childhood [1]. Up to the present day, the only curative therapy for βTM was the hematopoietic stem cell transplantation, which is available only in a limited set of patients, due to the age of the recipient and the lack of matching donors [1]. Gene therapy and editing approaches, as well as a new generation of experimental drugs, hold the promise for a definitive treatment for βTM [1–5]. Transfusion with packed RBCs (P-RBCs) remains the main therapeutic option for βTM patients
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