Budd-Chiari Syndrome in Patient with Primary ThrombocythaemiaTreated with Interferon Alpha Medically Managed by Transjugular Porto-systemic Shunt
Buddův-Chiariho syndrom u pacientky s primárnítrombocytémií léčené interferonem alfa řešenýtransjugulární portosystémovou spojkou
Mladá žena s anamnézou primární trombocytémie, léčená interferonem alfa, byla přijata na naši kliniku proprogredující bolesti břicha, hepatomegalii, ascites a alteraci jaterních funkcí. Zobrazovacími metodami byl prokázánsyndrom Budda-Chiariho, způsobený trombózou jaterních žil na podkladě zevního útlaku intrahepatální porce dolníduté žíly zvětšeným jaterním lalokem. Příčina syndromu není jasná, diferenciálně diagnosticky lze uvažovato orgánové infiltraci základní chorobou, možný je též primární či interferonem indukovaný antifosfolipidovýsyndrom či kombinace více faktorů. Definitivní příčinu by mohla stanovit jaterní biopsie, která nebyla provedenapro technické problémy.
Klíčová slova:
Buddův-Chiariho syndrom, primární trombocytémie, interferon alfa, antifosfolipidový syndrom,orgánová infiltrace, TIPS.
Authors:
L. Brunerová 5; H. Bartáková 1; M. Jankovská 2; B. Cieslarová 3; P. Hůlek 4; T. Kozák 2; M. Anděl 5
Authors‘ workplace:
II. interní klinika 3. LF UK a FNKV, Praha 2Oddělení klinické hematologie FNKV, Praha 3I. interní klinika 3. LF UK a FNKV, Praha 4II. interní klinika FN, Hradec Králové 5Laboratoř patofyziologie metabolizmu FNKV, Praha
1
Published in:
Čas. Lék. čes. 2004; : 198-201
Category:
Overview
The case report of a young female patient with personal history of primary thrombocythaemia, treated with interferonalpha, admitted to our medical department for severe abdominal pain, hepatomegaly, ascites and alteration of hepaticfunction is presented. Magnetic resonance imaging showed the picture typical for Budd-Chiari syndrome caused byexternal obstruction of the intrahepatal portion of inferior vena cava. The cause of the syndrome remains uncertain,possibility of the haematogenic infiltration of the liver or venal thrombosis within primary or secondary (interferon- induced) antiphospholipid syndrome is discussed. Liver biopsy could elucidate the exact cause, but it was notperformed for technical problems.
Key words:
Budd-Chiari syndrome, primary thrombocythaemia, interferon alpha, antiphospholipid syndrome,infiltration of organs, TIPS.
Labels
Addictology Allergology and clinical immunology Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric gastroenterology Paediatric surgery Paediatric cardiology Paediatric neurology Paediatric ENT Paediatric psychiatry Paediatric rheumatology Diabetology Pharmacy Vascular surgery Pain management Dental HygienistArticle was published in
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