Cardiac T2* MR in patients with thalassemia major: a 10-year long-term follow-up.

Abstract

The consequence of regular blood transfusion in patients with thalassemia major (TM) is iron overload. Herein, we report the long-term impact of chelation on liver iron concentration (LIC) and cardiac T2* MR in patients with TM. This is a retrospective cohort study over 10 years of adolescents and adults with TM aged at least 10 years who had their first cardiac T2* MR between September 2006 and February 2007. One-year chelation therapy was considered the unit of analysis. A total of 99 patients were included in this study with a median age of 18 years. The median cardiac T2* MR and LIC at baseline were 19 ms and 11.6 mg/g dw, respectively. During follow-up, 18 patients died and six underwent successful bone marrow transplantation. Factors associated with decreased survival were older age (HR 1.12, p = 0.014) and high risk cardiac T2* (HR 8.04, p = 0.004). The median cardiac T2* and LIC significantly improved over the 10-year follow-up period (p = 0.000011 and 0.00072, respectively). In conclusion, this long-term "real-life" study confirms that low cardiac T2* adversely impacts the overall survival in patients with TM. Higher baseline LIC predicts a larger reduction in LIC, and lower baseline cardiac T2* predicts a larger improvement in T2*.

• Authors: Shahina Daar, Murtadha Al Khabori, Sarah Al Rahbi, Moez Hassan, AbuBakr El Tigani, Dudley J Pennell
• Journal: Annals of hematology (Ann Hematol) Vol. 99 Issue 9 Pg. 2009-2017 (Sep 2020) ISSN: 1432-0584 [Electronic] Germany
• PMID: 32556452 (Publication Type: Journal Article)

Chemical References

• Iron Chelating Agents
• Deferiprone
• Deferasirox

Topics

• Adolescent
• Chelation Therapy (methods, trends)
• Cohort Studies
• Deferasirox (therapeutic use)
• Deferiprone (therapeutic use)
• Female
• Follow-Up Studies
• Humans
• Iron Chelating Agents (therapeutic use)
• Iron Overload (diagnostic imaging, drug therapy, mortality)
• Magnetic Resonance Imaging, Cine (methods)
• Male
• Retrospective Studies
• Survival Rate (trends)
• Time Factors
• Young Adult
• beta-Thalassemia (diagnostic imaging, drug therapy, mortality)