In this cross-sectional study, we compared the quality of life (QOL) in transfusion-dependent thalassemic patients who survived matched sibling hematopoietic SCT (HSCT, n ¼ 24) with patients treated conventionally with transfusion and iron chelation (n ¼ 74). WHOQOL-BREF(HK) and PedsQL questionnaires were administered to patients aged 418 years and 5-12 years, respectively. Patients aged 12-18 years received both questionnaires. WHOQOL-BREF(HK) revealed post transplant patients rated overall health better than those treated conventionally (score 3.67 vs 3.06, P ¼ 0.01). They are less dependent on medical aids (3.87 vs 2.96, P ¼ 0.006), having higher activity level (4.00 vs 3.36, P ¼ 0.026) and better personal relationships (4.13 vs 3.69, P ¼ 0.014). Physical health domain score was better (75.20 vs 63.94, P ¼ 0.007). These differences remained significant after adjustment for comorbidities. PedsQL revealed post transplant patients rated better for running (3.53 vs 2.72, P ¼ 0.001) and sports (3.20 vs 2.64, P ¼ 0.038), even after adjustment for comorbidities, but were less satisfied for school absence to attend hospital (2.53 vs 3.29, P ¼ 0.03). Post transplant patients were significantly more likely to consider marriage (100 vs 75.7%, P ¼ 0.033), but not childbearing (66.7 vs 51.4%, P ¼ 0.28). In conclusion, transplanted thalassemic patients enjoy better QOL, mainly in physical health, compared with conventionally treated patients. This information is important to patients considering HSCT.

The LV composite index of strain, torsion, and dyssynchrony derived from 3DSTE enables sensitive detection of myocardial iron overload in patients with thalassemia.

Our previous study showed that combined therapy with deferiprone (L1) and deferoxamine (DFO) was safe and efficacious in reducing iron overload in poorly-chelated thalassemia major patients for the short-term but the magnetic resonance imaging (MRI) T2* evaluation was not available at that time. Since October 2006, we applied a standardized chelation protocol by stratifying transfusion-dependent thalassemic patients into three groups, namely well-chelated group (A), poorly-chelated group without (B) or with (C) risk of cardiac complications, based on their serum ferritin (SF) levels and magnetic resonance imaging (MRI) cardiac T2* measurements. The patients in each group were given options of chelation regimens to improve their iron overload status. Chelation regimens included continuation or intensification of DFO alone (Regimen Ic or Ii, respectively), L1 alone (Regimen II), and combined therapy with L1 and DFO (Regimen III). Group A patients continued with Regimen Ic. Group B patients could opt for either Regimen Ii or II/III. Group C patients could opt for either Regimen Ii or III. Serum ferritin levels and MRI cardiac and liver T2* measurements were evaluated after 1 year of treatment. Fifty-seven patients (27 males, 30 females; age range 5-34 years, median: 25 years) were categorized into Group A (n = 3), B (n = 20) and C (n = 34). All Group A patients continued with DFO treatment. In Group B, seven were on Regimen Ii, five on Regimen II and five on Regimen III. In Group C, five were on Regimen Ii, two on Regimen II and 26 on Regimen III. Significant improvement was noted only for Group C patients using Regimen III (combined therapy) in SF levels, cardiac T2* and liver T2* measurements.