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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

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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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Acknowledgements

We would like to thank all the colleagues involved in the MIOT project (https://miot.ftgm.it/). We thank Claudia Santarlasci for her skillful secretarial work. We finally thank all patients for their cooperation.

Funding

The MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A. and ApoPharma Inc.).

Author information

Authors and Affiliations

  1. Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Naples, Italy

    Paolo Ricchi, Chrysanthos Grigoratos & Silvia Costantini

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

    Antonella Meloni, Laura Pistoia & Alessia Pepe

  3. Sezione di Scienze Radiologiche - Dipartimento di Biopatologia e Biotecnologie Mediche, Policlinico “Paolo Giaccone”, Palermo, Italy

    Patrizia Toia & Massimo Midiri

  4. Unità Operativa Complessa Diagnostica per Immagini, Ospedale “Sandro Pertini”, Rome, Italy

    Priscilla Fina & Paolo Preziosi

  5. Unità Operativa Complessa Ematologia con Talassemia, ARNAS Civico “Benfratelli-Di Cristina”, Palermo, Italy

    Zelia Borsellino

  6. Unità Operativa Dipartimentale Talassemia, Presidio Ospedaliero Garibaldi-Centro-ARNAS Garibaldi, Catania, Italy

    Roberto Lisi

  7. Servizio Microcitemia, Presidio Ospedaliero ASL 5, Crotone, Italy

    Mario Rocca

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  1. Paolo Ricchi
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Correspondence to Alessia Pepe.

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Ricchi, P., Meloni, A., Grigoratos, C. et al. 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. Ann Hematol 98, 1333–1339 (2019). https://doi.org/10.1007/s00277-019-03659-1

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