Splenomegaly and hypersplenism in hepatic vena cava syndrome

BACKGROUND AND AIM: Hepatic vena cava syndrome (HVCS) is a bacterial infection-induced obliterative disease of the inferior vena cava at the site of the hepatic vein openings that causes chronic liver disease with a high incidence of liver cirrhosis and a moderate incidence of hepatocellular carcinoma (HCC). HVCS typically manifests clinically as recurrent mild jaundice and/or a mild elevation of transaminases or ascites as a result of hepatic venous outflow obstruction. This study is an investigation of the complications and treatment results of a large cohort of HVCS patients with an emphasis on splenomegaly and hypersplenism. MATERIALS AND METHODS: A total of 1935 (1335 male and 600 female) patients with HVCS seen between 2004 and 2019 for splenomegaly and hypersplenism were enrolled in this retrospective study. The incidence of splenomegaly, ascites, cirrhosis, HCC, and other complications was measured and analyzed. RESULTS: Long-term follow-up of a large study group seen over several years indicated that 16% had mild splenomegaly, and 50% of these developed cytopenia commonly thrombocytopenia and/or leucopenia. CONCLUSION: The development of hypersplenism was related to recurrent or prolonged acute exacerbation of HVCS. This resulted in an increased incidence of ascites and cirrhosis as well as complications, such as hepatic encephalopathy, gastrointestinal or mucosal bleeding, or mortality.