Rupture of splenic artery aneurysm in a man with polycythemia vera and acquired von Willebrand syndrome

Splenic artery aneurysms (SAA) are the most common visceral artery aneurysms, with causes including pregnancy/multiparity, hypertension, portal hypertension, connective tissue disease, fibromuscular dysplasia and atherosclerosis.1–3 While the vast majority of SAA are asymptomatic, the rupture rate is 2%, with mortality as high as 36%.1 Sudden onset of left upper quadrant pain and hemodynamic instability is a prototypical presentation of rupture. PV is a myeloproliferative neoplasm associated with increased red blood cell mass and splenomegaly. Primary goals of management are to limit the risk of arterial and venous thrombosis using antiplatelet therapy, phlebotomy, interferon therapy or Jak1-2 inhibitors.4 Less common hemorrhagic complications have also been described in PV,5 related to underlying thrombotic events, antiplatelet medications or acquired von Willebrand syndrome.