Symptomatic splenomegaly in polycythemia vera: a review of the indications for splenectomy and perioperative considerations

Abstract

Polycythemia vera is a condition that surgeons do not commonly encounter. Advances in medical management have largely led to avoidance of surgical intervention in most patients. Indications and timing of splenectomy have been the subject of debate since the disease was first described in the late 19th century. Though anemia and thrombocytopenia associated with polycythemia vera only transiently respond to splenectomy, painful splenomegaly with infarction or compression of surrounding viscera are presently accepted indications for surgery. Special consideration must be given to polycythemia vera patients both preoperatively and postoperatively due to altered coagulation and anatomy. We present a review of the pathophysiology, medical treatment, indications for surgical intervention, and perioperative considerations for polycythemia vera.