Red blood cell consumption in a large cohort of patients with thalassaemia: a retrospective analysis of main predictors
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ORIGINAL ARTICLE
Red blood cell consumption in a large cohort of patients with thalassaemia: a retrospective analysis of main predictors Paolo Ricchi 1 & Antonella Meloni 2 & Silvia Costantini 1 & Anna Spasiano 1 & Patrizia Cinque 1 & Biagio Gargiulo 1 & Alessia Pepe 2 & Aldo Filosa 1 Received: 21 January 2020 / Accepted: 20 April 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract The phenotype/genotype relationship of patients with transfusion-dependent thalassaemia (TDT) is particularly complex and variable, thus generating different levels of severity and of annual transfusion volume (ATV). In this study, we explored the role and the contribution of several factors potentially involved in determining mean ATV in a cohort of TDT patients which have been followed since long time. We collected data on one-hundred and twenty-seven patients with transfusion-dependent βthalassaemia followed at Rare Blood Cell Disease Unit, AORN Cardarelli Hospital. Age at first transfusion, genotype, spleen status (splenectomy or not), and mean soluble transferrin receptor (sTfR) were the parameters included in the analysis. At stepwise regression analysis which included all the parameters, only splenectomy and mean sTfR significantly predicted the mean ATV (F = 70.94, P < 0.0001, R2 = 0.540). Overall, our data may suggest that in patients with TDT, the measurement of sTfR level together with the spleen status could contribute, more accurately than genotype, to provide a basal evaluation of residual erythropoietic activity and mean ATV. Keywords Thalassaemia . Biomarker . Genotype . Erythropoiesis
Introduction For long time, the standard of care for patients with transfusion-dependent thalassaemia (TDT) has been based on the lifelong administration of red blood cell transfusions in order to ameliorate anaemia and suppress ineffective and expanded erythropoiesis [1, 2]. Therefore, in these patients, iron overload occurs mainly as a consequence of transfusions. It is also becoming more and more clear that both an optimal transfusion and chelation therapy increase the survival of patients [3]. Although TDT patients are clearly different from those with non-transfusion-dependent thalassaemia (NTDT), within each group there may be great variability and some
* Paolo Ricchi [email protected] 1
U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Via A. Cardarelli 9, 80131 Naples, Italy
2
Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy
overlapping. Considering those with TDT, the majority of patients develops a severe form of anaemia (thalassaemia major) and is transfusion dependent from the first years of life depending on the severity of the disease. On the other hand, expanded erythropoiesis better characterizes patients with NTDT or thalassaemia intermedia [4], where the blood requirement is occasional or lower than that observed for TDT, but can increase as per clinical commitment. In fact, several patients with N
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