Acute Budd-Chiari syndrome, portal and splenic vein thrombosis in a patient with ulcerative colitis associated with antiphospholipid antibodies and protein C deficiency

Summary

We report the case of a female patient who had severe thrombotic complications in peripheral (V. jugularis, subclavia, brachialis, poplitea) and visceral (portal and splenic) veins 4 years after the first diagnosis of severe ulcerative pancolitis. A thrombolysis therapy for subclavian and jugular vein thrombosis was performed without complication, but she soon developed acute thrombosis of the hepatic veins (acute Budd-Chiari syndrome). She quickly recovered after liver transplantation and now - 6 years later - she lives a normal life with continuous anticoagulation and medical therapy of the colitis.

3 possible causes for the severe coagulation defect in this patient can be supposed: Thrombocytosis, protein C deficiency and an antiphospholipid antibody syndrome.

Budd-Chiari-Syndrom, Pfortader- und Milzvenenthrombose bei einer Patientin mit Colitis ulcerosa, Antiphospholipid-Antikörpern und Protein-C-Mangel. Fallbericht und Literaturübersicht

Wir berichten über eine Patientin mit schwerer Pancolitis ulcerosa, die nach 4-jähriger Krankheitsdauer akute Thrombosen verschiedener peripherer (V. jugularis, subclavia, brachialis, poplitea) und viszeraler (V. portae, V. lienalis) Venen entwickelte. Sie überstand komplikationslos eine Urokinase-Lysetherapie bei Thrombose der V. subclavia und V. jugularis, erkrankte dann aber an einem akuten Verschluss der Lebervenen (Budd-Chiari-Syndrom). Nach einer Lebertransplantation erholte sie sich rasch und führt jetzt - 6 Jahre später - unter kontinuierlicher Antikoagulation und medikamentöser Therapie der Kolitis ein normales Leben.

Als Ursache der schweren Gerinnungsstörung kommen eine Thrombozytose, ein Protein-C-Mangel und ein Antiphospholipid-Antikörpersyndrom infrage.

Key words

Budd-Chiari Syndrome - Portal Vein Thrombosis - Ulcerative Colitis - Protein C Deficiency - Antiphospholipid Antibodies - Thrombolysis - Liver Transplantation

Schlüsselwörter

Budd-Chiari-Syndrom - Pfortaderthrombose - Colitis ulcerosa - Protein-C-Mangel - Antiphospholid-Antikörper - Thrombolysetherapie - Lebertransplantation

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