Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis.

Hereditary spherocytosis consists of a group of haemolytic anaemias caused by defects in the proteins involved in the vertical interactions between the membrane skeleton and the lipid bilayer of the red blood cell. Inheritance is most commonly autosomal dominant with variable expression. Splenectomy may be indicated because of anaemia or for the prevention of gall-stones. We describe a patient who presented with symptoms of pulmonary hypertension 32 years after splenectomy. Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) have been associated with splenectomy, while chronic haemolysis may result in haemolysis-associated pulmonary hypertension. We briefly discuss the current views on the pathophysiology, diagnosis and management of this rare condition.