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. 2023 Oct 13;102(41):e35455.
doi: 10.1097/MD.0000000000035455.

Successful chelation in beta-thalassemia major in the 21st century

Christina Fragodimitri  1 , Vasiliki Schiza  1 , Anastasios Giakoumis  2 , Kalliopi Drakaki  1 , Anastasia Salichou  1 , Fotis Karampatsos  1 , Jacqueline Yousef  1 , Markissia Karageorga  1 , Vasili Berdoukas  3 , Athanasios Aessopos  4
Affiliations

Successful chelation in beta-thalassemia major in the 21st century

Christina Fragodimitri et al. Medicine (Baltimore). .

Abstract

This century has seen a revolution the management of beta-thalassemia major. Over a 12-year period to 2016, we aimed to analyze the benefits of such advances. In 209 patients, independent of the chelation regimen, ferritin, cardiac T2* and liver iron concentration changes were evaluated. We defined chelation success (ChS) as no iron load in the heart and acceptable levels in the liver. Over 3 early magnetic resonance imagings, the same parameters were assessed in 2 subgroups, the only 2 that had sufficient patients continuing on 1 regimen and for a significant period of time, 1 on deferrioxamine (low iron load patients n = 41, Group A) and 1 on deferoxamine-deferiprone (iron overloaded n = 60, Group B). Finally, 28 deaths and causes were compared to those of an earlier period. The 209 patients significantly optimized those indices, while the number of patients with chelation success, increased from 6% to 51% (P < .0001). In group A, ChS after about 8 years increased from 21 to 46% (P = .006), while in Group B, from 0% to 60% (P < .001) after about 7 years. Deaths over the 2 periods showed significant reduction. Combined clearance of cardiac and liver iron (ChS) is feasible and should become the new target for all patients. This requires, serial magnetic resonance imagings and often prolonged intensified chelation for patients.

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Conflict of interest statement

Vasili Berdoukas is a paid consultant to Chiesi Canada Inc. Chiesi is the distributor of the iron chelator deferiprone. The other authors have no conflict of interest to disclose.

Figures

Figure 1.
Figure 1.
(A–G) Showing graphically, the results of the studied parameters changes, between first and last MRI in the overall analysis. The median time interval in years is shown in the last column. MRI = magnetic resonance imaging.
Figure 2.
Figure 2.
Shows the percentage of the 209 patients on different chelation regimens at the median time of each of the 3 MRIs performed. MRI = magnetic resonance imaging.
Figure 3.
Figure 3.
(A–E) Showing graphically, the comparison of independent samples between Group A and Group B at consequent MRI studies. Median time interval in years for both groups between scans is shown is shown in figures (A–D) and the median time interval for each group is shown in (E). MRI = magnetic resonance imaging.
Figure 4.
Figure 4.
(A–B) showing graphically, the Comparison of two periods regarding causes of death in absolute number and percentage.
See this image and copyright information in PMC

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