Article Commentary: 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.

Spontaneous splenic rupture in polycythemia vera

“…The only indications in PV for which splenectomy is definitively therapeutic are painful splenomegaly and local compressive symptoms [13]. Complications of PV post-splenectomy include anemia, hyper viscosity and thrombosis (most commonly in Porto-mesenteric system of patients with hematological indications), increased risk of cancer, infection (especially encapsulated bacteria), platelet dysfunction, thrombocytosis, portal hypertension, and acquired von Willebrand deficiency (platelets above one million lead to loss of large vWF multimers in plasma and cause bleeding diathesis, which is reversible with desmopressin or platelet reduction) [13]. Infections and thromboembolic events cause the most medical morbidity postsplenectomy, they are more common in the immediate postoperative period but persist throughout life.…”

“…Infections and thromboembolic events cause the most medical morbidity postsplenectomy, they are more common in the immediate postoperative period but persist throughout life. Management includes antibiotics, anticoagulation and antiplatelet therapy as clinically indicated, patient education, and vaccinations [13]. Patients who have good long-term control of hematocrit are less likely to have complications than those with short-term or poor control.…”

The Havoc of a Giant Spleen

“…Learning points from this case can be especially emphasized from the management of the complications of her splenectomy in relation to her reactive thrombocytosis and multivessel thrombosis. Splenectomy in patients with various hematologic has been associated with an increased incidence of vascular complications, including venous and arterial thrombosis and pulmonary hypertension [6,10]. The marked thrombocytosis following splenectomy was thought to be the main contri0butor of her multi-vessel thrombosis.…”

“…Common complications of thrombocytosis are thrombosis and hemorrhage [5]. Post-splenectomy venous thrombosis happens most commonly when platelet counts reach 600-800 K/µL and occurs in approximately 5% of patients [6].…”