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20.03.2019 | Original Article

Prevalence of extramedullary hematopoiesis, renal cysts, splenic and hepatic lesions, and vertebral hemangiomas among thalassemic patients: a retrospective study from the Myocardial Iron Overload in Thalassemia (MIOT) network

verfasst von: Paolo Ricchi, Antonella Meloni, Chrysanthos Grigoratos, Patrizia Toia, Priscilla Fina, Laura Pistoia, Silvia Costantini, Zelia Borsellino, Roberto Lisi, Mario Rocca, Paolo Preziosi, Massimo Midiri, Alessia Pepe

Erschienen in: Annals of Hematology | Ausgabe 6/2019

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Abstract

We determined the prevalence of incidental extracardiac findings (IEF) at Magnetic Resonance Imaging (MRI) potentially related to anemia and hypoxia in age- and sex-matched populations (N = 318) with thalassemia major (TM) and thalassemia intermedia (TI) enrolled in the Myocardial Iron Overload in Thalassemia network. Overall, IEFs were detected in 33.3% and 25.8% of patients with TI and TM, respectively (P = 0.114). TI and TM patients had elevated but comparable prevalence of renal, splenic and liver cysts, and vertebral hemangiomas while TI patients had a significant higher frequency of extramedullary hematopoiesis (EMH) (15.1% vs 4.4%; P = 0.002). The prevalence of total IEFs increased with advancing age. TI non-transfusion-dependent patients had a significantly lower frequency of renal cysts than TI transfusion-dependent patients (8.8% vs 26.4%; P = 0.005). The prevalence of renal cysts in the thalassemic population was significantly higher than that in the general population (19.2% vs 1.9%; P < 0.0001). Our data on renal cysts indicate a significant higher prevalence of these IEFs compared to the general population, suggesting the role of the inappropriate activation of the hypoxia-inducible factor system linked to the chronic hypoxia. The significant prevalence of IEF in thalassemia patients undergoing MRI for iron quantification should prompt the discussion of the inclusion of IEF in the MRI report.

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Titel: Prevalence of extramedullary hematopoiesis, renal cysts, splenic and hepatic lesions, and vertebral hemangiomas among thalassemic patients: a retrospective study from the Myocardial Iron Overload in Thalassemia (MIOT) network
verfasst von: Paolo Ricchi
Antonella Meloni
Chrysanthos Grigoratos
Patrizia Toia
Priscilla Fina
Laura Pistoia
Silvia Costantini
Zelia Borsellino
Roberto Lisi
Mario Rocca
Paolo Preziosi
Massimo Midiri
Alessia Pepe
Publikationsdatum: 20.03.2019
Verlag: Springer Berlin Heidelberg
Erschienen in: Annals of Hematology / Ausgabe 6/2019
Print ISSN: 0939-5555
Elektronische ISSN: 1432-0584
DOI: https://doi.org/10.1007/s00277-019-03659-1

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