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Red blood cell consumption in a large cohort of patients with thalassaemia: a retrospective analysis of main predictors.
Ricchi, Paolo; Meloni, Antonella; Costantini, Silvia; Spasiano, Anna; Cinque, Patrizia; Gargiulo, Biagio; Pepe, Alessia; Filosa, Aldo.
Afiliación
  • Ricchi P; U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Via A. Cardarelli 9, 80131, Naples, Italy. pabloricchi@libero.it.
  • Meloni A; Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
  • Costantini S; U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Via A. Cardarelli 9, 80131, Naples, Italy.
  • Spasiano A; U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Via A. Cardarelli 9, 80131, Naples, Italy.
  • Cinque P; U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Via A. Cardarelli 9, 80131, Naples, Italy.
  • Gargiulo B; U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Via A. Cardarelli 9, 80131, Naples, Italy.
  • Pepe A; Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
  • Filosa A; U.O.S.D. Malattie rare del globulo rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Via A. Cardarelli 9, 80131, Naples, Italy.
Ann Hematol ; 99(6): 1209-1215, 2020 Jun.
Article en En | MEDLINE | ID: mdl-32363417
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 ß-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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Esplenectomía / Talasemia beta / Transfusión de Eritrocitos Tipo de estudio: Diagnostic_studies / Etiology_studies / Incidence_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Aged / Child / Female / Humans / Male / Middle aged Idioma: En Revista: Ann Hematol Asunto de la revista: HEMATOLOGIA Año: 2020 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Alemania
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Esplenectomía / Talasemia beta / Transfusión de Eritrocitos Tipo de estudio: Diagnostic_studies / Etiology_studies / Incidence_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Aged / Child / Female / Humans / Male / Middle aged Idioma: En Revista: Ann Hematol Asunto de la revista: HEMATOLOGIA Año: 2020 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Alemania