Modern management of iron overload in thalassemia major patients guided by MRI techniques: real-world data from a long-term cohort study

• Published in: Annals of hematology Vol. 101; no. 3; pp. 521 - 529
• Main Authors: Bayraktaroglu, Selen, Karadas, Nihal, Onen, Sebnem, Karapinar, Deniz Yılmaz, Aydinok, Yesim
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.03.2022; Springer Nature B.V
• Subjects: Adolescent; Adult; beta-Thalassemia - complications; Blood diseases; Chelation therapy; Chelation Therapy - methods; Cohort analysis; Cohort Studies; Deferasirox - therapeutic use; Disease Management; Female; Hematology; Humans; Iron Chelating Agents - therapeutic use; Iron Overload - complications; Iron Overload - therapy; Liver; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; Male; Medicine; Medicine & Public Health; Middle Aged; Oncology; Original Article; Retrospective Studies; Trends; Young Adult; Iron overload; Iron chelation; Thalassemia major; Magnetic resonance imaging; Cardiac iron; Liver iron
• ISSN: 0939-5555; 1432-0584; 1432-0584
• DOI: 10.1007/s00277-021-04748-w

Monitoring liver and cardiac iron stores by magnetic resonance imaging (MRI) enables identifying patients at risk of organ-specific morbidity and better tailoring of iron chelation therapy in thalassemia. Nevertheless, serum ferritin (SF) remains the only tool for monitoring iron status in most resource-poor regions. In this study, we assessed the impact of using MRI techniques to guide iron chelation therapy on iron overload outcomes in a cohort of 99 patients with thalassemia major (TM, mean age at baselines 20.7 ± 6.9 years) followed from 2006 to 2019. We also assessed the ability of SF trends to predict changes in consecutive liver iron concentration (LIC) and cardiac T2* (cT2*) measurements. The most commonly used chelator was deferasirox at baseline (65%) and final (72%) assessments. Overall, patients with safe LIC values (< 7 mg/g dw) increased from 57 to 77%, and safe cT2* values (> 20 ms) increased from 72 to 86%. We obtained the most significant improvement in patients with severe and moderate liver ( p  = 0.006 and p  < 0.001) and cardiac ( p  < 0.0013 and p  < 0.0001) iron overload at baseline. SF trends were in the same direction in 64% of changes in LIC, but only 42% of changes were proportional. Most of the changes in SF (64%) and LIC (61%) could not predict changes in cT2*. Moreover, downward trends in SF and LIC were associated with worsening cardiac iron in 29% and 23.5% of consecutive cT2* measurements. Liver and cardiac MRI-driven oral iron chelation improved the iron status of subjects with TM and demonstrated the importance of using validated MRI techniques in critical clinical decisions.