[Budd-Chiari syndrome in a patient with paroxysmal nocturnal haemoglobinuria].

HISTORY AND CLINICAL FINDINGS: A 61-year-old man with dyspnea and diffuse abdominal pain due to increasing ascites caused by liver cirrhosis of unknown etiology was admitted for consideration of transjugular intrahepatic portosystemic stent-shunting (TIPSS). The patient's medical history included paroxysmal nocturnal hemoglobinuria (PNH), presenting as slight hemolysis diagnosed 24 years previously. One year before the patient underwent radical retropubic prostatectomy for a localized prostate cancer. Shortly after this intervention he developed ascites.

INVESTIGATIONS: Color Doppler ultrasonography revealed an abnormal flow in the major hepatic veins. Transjugular liver biopsy indicated hepatic a circulatory disorder. Hepatic venography revealed the so-called "spider web" pattern characteristic for the Budd-Chiari syndrome. The hypercoagulable state due to paroxysmal nocturnal hemoglobinuria was accentuated by manipulation on the prostate during prostatectomy and presumably resulted in a thrombotic obstruction of the hepatic veins.

TREATMENT AND CLINICAL COURSE: After exclusion of contraindications a transjugular intrahepatic portosystemic stent shunt (TIPSS) was performed, which led to a decrease of portal pressure. Signs of portal hypertension such as esophageal varices and ascites resolved completely. The patient has been free of complaints for one year.

CONCLUSION

We assume that a hypercoagulopathy due to asymptomatic paroxysmal nocturnal hemoglobinuria resulted in Budd-Chiari syndrome when boosted by postoperative release of procoagulation factors in the thrombokinase-rich prostate. TIPSS is a therapeutic option in these patients.