Higher hemoglobin is better in thalassemia

In this issue of Blood, Musallam, Forni, and colleagues show that survival in transfusion dependent beta-thalassemia (TDT) is measurably better in patients whose pretransfusion hemoglobin levels are maintained >10.5 g/dL, at the upper end of the currently recommended treatment range.(1) In this landmark study, the 10-year survival of a group of 779 patients with TDT of median age 33.1 years (range 18.1-61 years) increased monotonically from 91% to 100% in 5 categories marked by median pretransfusion hemoglobin levels that increased from <9.0 to >= 10.5 g/dL in 0.5-g/dL increments. Of note, 88% of the thalassemia-related deaths were in groups with a median hemoglobin <10 g/dL and 70% were from cardiovascular disease. When the data were stratified by ferritin, the association with hemoglobin level groups was only significant for ferritin <1000 ng/mL, consistent with an effect of anemia on survival separate from that of iron overload.