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Red blood cell consumption in a large cohort of patients with thalassaemia: a retrospective analysis of main predictors

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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 β-thalassaemia 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.

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Acknowledgments

We thank all patients for their cooperation.

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Authors and Affiliations

  1. U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Via A. Cardarelli 9, 80131, Naples, Italy

    Paolo Ricchi, Silvia Costantini, Anna Spasiano, Patrizia Cinque, Biagio Gargiulo & Aldo Filosa

  2. Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

    Antonella Meloni & Alessia Pepe

Authors
  1. Paolo Ricchi
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  2. Antonella Meloni
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  3. Silvia Costantini
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  4. Anna Spasiano
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  5. Patrizia Cinque
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  6. Biagio Gargiulo
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  7. Alessia Pepe
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  8. Aldo Filosa
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Corresponding author

Correspondence to Paolo Ricchi.

Ethics declarations

The study complied with the Declaration of Helsinki. All patients gave written informed consent to the protocol. The study was approved by the Ethics Committee of the Cardarelli-Santobono-Pausilipon Hospital, Napoli, Italy.

Conflict of interest

A.P. is the PI of the MIOT project that receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A., ApoPharma Inc. and Bayer). The remaining authors have nothing to disclose.

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Ricchi, P., Meloni, A., Costantini, S. et al. Red blood cell consumption in a large cohort of patients with thalassaemia: a retrospective analysis of main predictors. Ann Hematol 99, 1209–1215 (2020). https://doi.org/10.1007/s00277-020-04047-w

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  • DOI: https://doi.org/10.1007/s00277-020-04047-w

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