Long-term chelation therapy with deferasirox: effects on cardiac iron overload measured by T2* MRI.

We report the long-term effects of deferasirox 10-30 mg/kg/day on cardiac iron overload in a case series of five patients with transfusion-dependent beta-thalassaemia major who underwent up to 5 years of chelation therapy. Iron overload was monitored by multislice multi-echo T2* magnetic resonance imaging (MRI). Overall, T2* MRI showed a decrease from baseline in cardiac iron levels in all patients during treatment with deferasirox (baseline T2* levels 13-24 ms; final T2* levels 21-41 ms).

Hb Hinwil [beta38(C4)Thr-->Asn, ACC>AAC] associated with beta0-thalassemia in a Sicilian child.

Hemoglobins (Hbs) with high oxygen affinity play a well-known role among the causes of erythrocytosis. In 1996, a new Hb called Hb Hinwil or beta38(C4)Thr-->Asn was described. In carriers, it causes an increase in the number of red blood cells, total Hb, and hematocrit.