To the Editors:
At present, it is estimated that 10,000 to 20,000 patients in the USA are treated with long-term blood transfusion [1], including patients with sickle cell disease (SCD) who are often transfused to prevent stroke [2, 3]. Iron overload is common in patients with recurrent transfusion, and the degree of iron overload may correlate with the rate of transfusions [4, 5]. While iron overload and cirrhosis have not been definitively linked in patients with SCD, >11% of patients with SCD were found to have cirrhosis on autopsy and a possible link has long been suggested [6].
Hepatopulmonary syndrome (HPS) is a common pulmonary complication of cirrhosis, occurring in 5–32% of patients with cirrhosis [7]. HPS is pathologically characterised by the formation of dilated pulmonary capillaries or, less often, microscopic pleural and pulmonary arteriovenous communications [8]. Clinically, it is characterised by progressive hypoxaemia that often reverses following liver transplantation [6].
Patients with SCD are predisposed to complications of the pulmonary parenchyma and pulmonary vasculature, which often lead to chronic hypoxaemia. Well-described complications include pulmonary arterial hypertension, pulmonary venous hypertension, fat emboli, acute chest syndrome and pulmonary fibrosis [9]. However, to our knowledge, HPS associated with …