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


METHODS
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.


METHODS
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.


CONCLUSIONS
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.
Balta
Z
Z
Medizinische Klinik und Poliklinik I, Allgemeine Innere Medizin, Bonn.
Nattermann
J
J
Flacke
S
S
Sauerbruch
T
T
D001201:Ascites / Q000209:etiology; D001706:Biopsy / Q000379:methods; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D004932:Esophageal and Gastric Varices / Q000209:etiology; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000601:surgery; D006503:Hepatic Veins / Q000000981:diagnostic imaging; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D008099:Liver / Q000098:blood supply / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010690:Phlebography; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D011468:Prostatectomy / Q000009:adverse effects; D011471:Prostatic Neoplasms / Q000601:surgery; D018615:Ultrasonography, Doppler, Color