a single transfusion will raise liver iron by 1mg/g
the body can only eliminate the equivalent of one transfusion a year
hemolysis can be thought to be slightly protective of iron overload as the iron is cleared in urine via the kidneys
liver iron deposition is dependent on the average dose of chelator over time
however, cardiac iron deposition is dependent on time spent chelated
the consequence is that cardiac iron deposition can occur even with an adequate average dose of chelator titrated for prevention of hepatic iron loading
any cardiac T2* less than 10ms is a risk factor for developing heart failure
iron cardiomyopathy is completely reversible if the organs can be supported long enough
there is suggestion that calcium channel blockade can be cardioprotective from this standpoint
BID dosing of deferoxamine may be better than daily dosing
splenectomy is a significant risk factor for pulmonary hypertension
pulmonary hypertension results from abnormal mechanical forces and factors related to vasoconstriction that may result from ineffective erythropoiesis
many patients may also have restrictive and fibrotic lung disease
tricuspid regurgitation is a risk of pHTN in thalassemia and should be assessed every 1-3 years by echocardiogram
chronic transfusion therapy can reverse pulmonary hypertension
sildenafil can be considered for additional pulmonary hypertension management refractory to chronic transfusions
HFpEF is an emerging complication of long-term thalassemia; risks include arrhythmias and prior iron overload
thalassemia appears to age the heart by 10-15 years particularly if control of iron is suboptimal
proximate mechanisms of HFpEF include inflammation, insulin resistance, and fibrosis
by the time the heart is loaded, there is presumed iron-mediated damage to the pituitary gland and pancreas
pancreas MRI screening is the “canary in the coalmine” in the sense that a clean pancreas implies a clean heart; iron deposition in the pancreas warrants closer cardiac MRI screening
thalassemia intermedia carries a higher risk of pulmonary hypertension than thalassemia major