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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.
POLYCYTHEMIA VERA (PV) is a condition encountered infrequently by the surgeon because it is traditionally managed with a fair degree of success medically. However, under certain circumstances, such as painful splenic infarction or symptoms of compression, a surgeon's consultation may be requested. It is imperative that the consultant be aware of the multitude of pitfalls accompanying surgical intervention and the most appropriate means to avoid them in this unique population.
Definition
Polycythemia vera, one of the chronic myeloproliferative disorders, is characterized by an erythroid dominant, trilineage proliferation of red cells, white cells, and platelets, as well as their precursor cells.1 Conventionally, it is defined by two indistinct phases: a proliferative polycythemic phase transitioning into a postpolycythemic or "spent" phase. The former is characterized by an increased red cell mass and excessive proliferation of erythroid, myeloid, and megakaryocyte elements whereas the latter by progressive bone marrow fibrosis and failure, associated with extramedullary hematopoiesis, and acute leuke- mic transformation.2 Early disease symptoms are a consequence of overproduction of red blood cells, platelets, or both and include thrombosis, hemorrhage, pruritis, erythromelalgia, peptic ulcer disease, and ocular migraines.2^1 These symptoms may abate in the latter stages of the disease as the bone marrow is exhausted and myelofibrosis predominates, and are replaced by signs of anemia, thrombocytopenia, sys- temic manifestations, such as, fever and weight loss, and extramedullary hematopoiesis. Splenomegaly may develop in the early stages of the disease due to excessive sequestration or in the later stages due to its role in extramedullary hematopoiesis, potentially resulting in pain from capsular stretch or parenchymal infarct...