What is the role of surgery in the treatment of polycythemia vera (PV)?

Updated: Sep 20, 2021
  • Author: Srikanth Nagalla, MD, MS, FACP; Chief Editor: Sara J Grethlein, MD, FACP  more...
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Consider splenectomy in patients with painful splenomegaly or repeated episodes of thrombosis causing splenic infarction.

Budd-Chiari syndrome occurs in patients with myeloproliferative disease (MPD) and most frequently in young women. Surgical approaches to the management of Budd-Chiari syndrome are, therefore, relevant to patients with polycythemia vera. [37]

Budd-Chiari syndrome is a liver-related condition associated with large-vessel thromboses and outflow obstruction with inferior vena cava or portal vein thrombosis. This is associated with the development of ascites, hepatosplenomegaly, abdominal pain, and gastrointestinal bleeding, but 20% of patients are asymptomatic.

The diagnosis is made by using ultrasonography to identify portal vein patency. In addition to the standard computed tomography (CT) scan and magnetic resonance imaging (MRI), patients with Budd-Chiari syndrome may need invasive angiographic imaging to determine the hemodynamics of the liver and the intrahepatic and vena caval gradients to determine the best surgical procedure. The histology of the liver helps determine the acuteness of the problem, the presence of chronic changes, and the degree of cirrhosis. This determines whether a patient requires a shunt or a liver transplant.

The following procedures have been used in patients with Budd-Chiari syndrome:

  • Transjugular intrahepatic portosystemic shunt (TIPS)

  • Side-to-side portocaval shunt or mesocaval shunt, portocaval/cavoatrial shunt, or mesoatrial shunt

These procedures have been reported to be successful in 38-100% of patients, with follow-up ranging from 9-98 months.

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