Quantitative Susceptibility Mapping (QSM) to Guide Iron Chelating Therapy

Summary

The overall goal of this research is to help develop a new magnetic resonance (MR) method, Quantitative Susceptibility Mapping (QSM), to improve the measurement of liver iron concentrations without the need for a liver biopsy. Measurement of liver iron is important to diagnose and treat patients who have too much iron in their bodies (iron overload). Liver iron measurements by current MRI methods (R2 and R2\*) can be inaccurate because of the effects of fat, fibrosis and other abnormalities. QSM should not be affected by these factors and should be free of these errors. In this study, MRI measurements (QSM, R2 and R2\*) of iron in patients before liver transplant will be compared with chemical analysis of iron in liver explants (livers removed from patients undergoing liver transplant). The liver explants would otherwise be discarded. Investigators expect that this study will show that the new MRI method, QSM, is superior to the current MRI methods, R2 and R2\*.

Eligibility

Inclusion Criteria:

* Established diagnosis of thalassemia major
* Treatment with deferasirox formulated as Jadenu® as the sole iron chelating therapy (ICT)
* Regular transfusion with records maintained in the Cornell Thalassemia Program
* 2 years of age or older
* Females who are not pregnant

Inclusion Criteria (for healthy subjects):

* Men and women aged 21 years or older
* Able and willing to give consent
* No known hematological and liver disease
* No contraindications for MRI

Exclusion Criteria:

* A history of auditory or ocular toxicity related to ICT
* A history of poor adherence to prescribed therapy
* An inability to tolerate MRI examinations
* Treatment for mental illness
* Institutionalization or imprisonment

Conditions2

Locations2 sites

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