Hepatic vein thrombosis complicating polycythemia vera. Successful treatment with a portacaval shunt

Abstract

THROMBOTIC complications of polycythemia vera are common. In an autopsy series of 250 patients with polycythemia vera, Chievitz and Thiede reported an incidence of thrombotic complications in 63%.¹ Death was attributed to thrombosis in 40% of the entire series. The hepatic veins are a rare site of spontaneous thrombosis in polycythemia vera.^2,3 Parker, in reviewing the world literature on hepatic vein occlusion (Budd-Chiari syndrome), accumulated 149 case reports in which the diagnosis had been proven at postmortem examination, and added 15 cases of his own.⁴ Although polycythemia vera was the most common cause of hepatic vein thrombosis in those patients in whom the etiology had been established, he could find only 17 cases. Subsequent to his review, there have been only a few additional reports in the literature of similar cases. The case presented below is that of a patient who developed sudden hepatic vein thrombosis as