Association of polycythemia vera with non-cirrhotic portal hypertension in five patients: A case series

Polycythemia vera (PV) and non-cirrhotic portal hypertension (NCPH) are relatively independent diseases, and few studies have linked them. However, in clinical settings, there may be a causal relationship. The aim of the present study was to analyze the clinical data of five patients with portal hypertension caused by PV and summarize the characteristics of PV with portal hypertension, to enhance the knowledge of this disease. The clinical data of five patients with PV and portal hypertension treated at Beijing You'an Hospital (Beijing, China) from January 2010 to March 2022 were retrospectively collected. The characteristics of these patients were then summarized and analyzed, including general information, laboratory tests, imaging and gastroscopy data. Overall, four patients were diagnosed with PV earlier compared with those with NCPH (ranging between days and years), whereas one patient was diagnosed with NCPH at the time of PV diagnosis. These four patients had blood cell elevations of 2-3 categories (red blood cells, white blood cells or platelets). The Child classification of liver functions in all five patients were found to be grades A-B. All five patients had splenomegaly, where three patients had portal vein thrombosis and cavernous degeneration. In addition, four patients had moderate or severe esophageal varices. In conclusion, to the best of our knowledge, this was the first case series of NCPH caused by PV. Among the patients, it was revealed that: i) NCPH caused by PV had milder liver function damage compared with cirrhosis-induced portal hypertension; ii) splenomegaly, ascites and esophageal varicose veins were prominent symptoms of NCPH caused by PV; iii) If PV is diagnosed, esophagogastroduodenoscopy should be performed as early as possible and regularly, where primary prevention measures for esophageal variceal hemorrhage are recommended; and iv) patients with PV with portal hypertension are at risk of thrombosis and bleeding, but it remains to be determined whether early antithrombotic therapy can reduce complications.