Magnetic resonance imaging during management of patients with transfusion-dependent thalassemia: a single-center experience.

BACKGROUND: Cardiac and hepatic magnetic resonance imaging evaluation during treatment can tailor physicians' chelation therapy titrations. AIM: The aim of the study was to assess the relationship of cardiac and hepatic T2* values with chelation therapy in patients with transfusion-dependent thalassemia (TDT). METHODS: A total of 106 patients with TDT who were followed up in Istanbul Medical Faculty Thalassemia Center were evaluated for the study. Forty-eight (45%) patients with TDT had more than one consecutive MRI examination. The patients were divided into three subgroups according to the cardiac T2* values as the high-risk group (T2* MRI < 10 ms), medium-risk group (T2* MRI 10-20 ms), and the low-risk group (T2* MRI > 20 ms). RESULTS: The majority of patients used DFX (deferasirox) (79%) and deferiprone (DFP) (17%). Approximately 80% of patients according to cardiac T2* value and 40% of patients according to hepatic T2* value were initially in the low-risk group. Patients with follow-up MRI examinations exhibited significant improvement in liver iron concentration, which correlated with an increase in hepatic T2* values. The decrease of liver iron concentration was prominent in the DFX group (p < 0.01). The serum ferritin level was significantly correlated with liver iron concentrations (rs = 0.65, p < 0.001), hepatic T2* value (rs = - 0.62, p < 0.001), but not with cardiac T2* value (rs = - 0.20, p = 0.07). CONCLUSION: Cardiovascular and hepatic MRI is a useful follow-up tool during the assessment of risk groups and chelation therapy of patients with TDT. Consecutive MRI tests showed good monitoring of cardiac and liver iron overload.

Olfactory dysfunction and quality of life in patients with transfusion-dependent thalassemia.

Transfusion-dependent thalassemia (TDT) is a group of thalassemia syndromes that require regular blood transfusions for survival. It is unknown whether the sense of smell of patients with TDT is affected, and if so, whether smell loss has an adverse effect on quality of life (QOL). Olfactory thresholds were measured using Snap & Sniff® wands. QOL was assessed via the Short Form-36 (SF-36) questionnaire. Forty-two TDT patients from the Thalassemia Center in Istanbul Medical Faculty were tested (mean age 26.6 years), along with 42 healthy controls (mean age 28.0 years). Mean olfactory sensitivity was lower in the TDT patients than the controls (p < 0.0001). TDT was associated with lower mean QOL scores on the domains of physical function (p < 0.0001), physical role limitation (p = 0.011), and general health (p < 0.0001). Within the TDT group, significant correlations were present between the threshold scores and physical function, physical role limitation, emotional role limitation, and general health. Patients with TDT are less sensitive to odors than healthy controls and the lower olfactory test scores are related to lower quality of life, suggesting that decreased smell function is an additional complication of this disease.