Paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome: therapeutic challenge with bone marrow transplantation, transjugular intrahepatic portosystemic shunt, and vena cava stent.

Stenosis of inferior vena cava can be a cause of or a consequence of Budd-Chiari syndrome (BCS). However, its occurrence after transjugular intrahepatic portosystemic shunt (TIPS) insertion has only been twice reported. We report the case of a 23-year-old man who presented BCS of the three suprahepatic veins. The cause of BCS was paroxysmal nocturnal hemoglobinuria, which was treated by bone marrow transplantation. A few months later, it was necessary to insert a TIPS because of refractory ascites and severe denutrition. However, refractory ascites was persistent and esophageal varices bleeding occurred. A TIPS desobstruction was needed, and during this angiography a stenosis of the suprahepatic vena cava was found and was treated with dilatation and stent. In a few weeks, the patient's clinical and nutritional state improved and, after 9 months hospitalization, the patient was discharged. One year later, this patient had no symptoms with an excellent permeability of TIPS and vena cava stent. This case report confirms that TIPS is a good treatment for BCS, but stenosis of inferior vena cava can occur after such a treatment.