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Etiology and Clinical Outcome of Budd-Chiari Syndrome and Portal Vein Thrombosis

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Etiology and Clinical Outcome of Budd-Chiari Syndrome and Portal Vein Thrombosis Etiology and Clinical Outcome of Budd-Chiari Syndrome and Portal Vein Thrombosis Jildou Hoekstra ISBN: 978-90-8559-172-6 Financial support for printing of this thesis was kindly provided by the Department of Gastroenterology and Hepatology of the Erasmus University Medical Center, J.E. Jurriaanse Stichting and the Dutch Society of Hepatology (NVH). Printed by: Optima Grafische Communicatie, Rotterdam, the Netherlands Cover: photograph & design by Niels Agatz © 2010. Jildou Hoekstra, Rotterdam, the Netherlands. All rights reserved. No parts of this work may be reproduced or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior permission of the author. Etiology and Clinical Outcome of Budd-Chiari Syndrome and Portal Vein Thrombosis Etiologie en klinisch beloop van Budd-Chiari syndroom en vena portae trombose Proefschrift ter verkrijging van de graad van doctor aan de Erasmus Universiteit Rotterdam op gezag van de rector magnificus Prof.dr. H.G. Schmidt en volgens besluit van het College voor Promoties. De openbare verdediging zal plaatsvinden op vrijdag 17 december 2010 om 11:30 uur door Jildou Hoekstra geboren te Amstelveen PROMOTIECOMMISSIE Promotoren: Prof.dr. H.L.A. Janssen Prof.dr. F.W.G. Leebeek Overige leden: Prof.dr. H.J. Metselaar Prof.dr. P. Sonneveld Prof.dr. J.P.H. Drenth sin en wille kin folle tille CONTENTS Chapter 1 Introduction 9 Chapter 2 Etiologic factors underlying Budd-Chiari syndrome and portal vein 35 thrombosis: clues for site-specific thrombosis Chapter 3 Impaired fibrinolysis as a risk factor for Budd-Chiari syndrome 51 Chapter 4 Proteomic analysis reveals that apolipoprotein A1 levels are 69 decreased in patients with Budd-Chiari syndrome Chapter 5 Paroxysmal nocturnal hemoglobinuria in Budd-Chiari syndrome: 85 findings from a cohort study Chapter 6 Effect of anticoagulation therapy in patients with non-cirrhotic 105 non-malignant extrahepatic portal vein thrombosis Chapter 7 Long-term follow-up of patients with portal vein thrombosis and 119 myeloproliferative neoplasms Chapter 8 Pregnancy in women with portal vein thrombosis: results of a 133 multicentric European study on maternal and foetal management and outcome Chapter 9 General discussion and conclusions 145 Summary 161 Samenvatting 165 List of publications 171 PhD Portfolio summary 173 Dankwoord 175 Curriculum Vitae 179 Chapter 1 Introduction J. Hoekstra, H.L.A. Janssen Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands Adapted from: Vascular Liver Disorders (I): Diagnosis, Treatment and Prognosis of Budd-Chiari Syndrome. Neth J Med 2008;66:334-9. Vascular Liver Disorders (II): Portal Vein Thrombosis. Neth J Med 2009;67:46-53. Introduction VASCULAR LIVER DISORDERS The liver receives approximately one-third of the resting cardiac output. Blood flow to the liver is supplied by both an arterial (hepatic artery) and a venous (portal vein) system and Chapter 1 three hepatic veins provide drainage of blood from the liver to the inferior vena cava. The hepatic vascular system is quite dynamic and has the ability to function as a reservoir for blood within the general circulation. Different conditions can interfere with hepatic blood flow and cause disease. The most important clinical syndrome affected by obstruction within the liver vasculature is portal hypertension. Portal hypertension is defined by an increase in the pressure of the portal venous system which results from a disruption of normal blood flow at either a prehepatic, intrahepatic or posthepatic level. The most common cause of portal hypertension in the Western world is liver cirrhosis, leading to an elevated portal pres- sure due to an increased resistance to intrahepatic blood flow as a result of architectural distortion of the liver. In the absence of liver cirrhosis, numerous less common disorders are known to cause, so- called, non-cirrhotic portal hypertension. Two rare diseases, characterized by thrombosis of the large hepatic vessels are Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT). Both these disorders share certain features, such as etiologic factors causing thrombosis and the development of portal hypertension, but are considered as separate disease entities based on the location of venous obstruction and their variable clinical presentation. BCS is defined as an obstruction of the hepatic venous outflow tract, ranging from the level of the small hepatic veins up to the junction of the inferior vena cava with the right atrium. Most cases of BCS in the Western world are caused by thrombosis of the hepatic veins, sometimes in combination with thrombosis of the inferior vena cava. The exact incidence of BCS is unknown but is estimated around 1 per million. Thrombotic occlusion of the portal vein is somewhat more common, especially as a complication in patients with liver cirrhosis. Non- cirrhotic PVT has a diverse etiology but a significantly better outcome than in patients with underlying liver cirrhosis or hepatobiliary malignancies. BUDD-CHIARI SYNDROME Budd-Chiari syndrome (BCS), first described in 18461 , is a rare clinical entity resulting from an occlusion of the hepatic venous outflow tract. Hepatic outflow obstruction related to right- sided cardiac failure or sinusoidal obstruction syndrome (SOS, also known as veno-occlusive disease 2) is not included in the definition of BCS. In Europe and North America, the main cause of outflow obstruction is thrombosis of the hepatic veins. 3 Involvement or isolated obstruction of the inferior vena cava is encountered relatively more often in Asian countries, such as India and Japan. 4-5 Over the past years, improved imaging techniques and new 11 Chapter 1 insights into potential causative factors have significantly contributed to the diagnosis and treatment of BCS. Nevertheless, due to the rarity of this disorder, most existent knowledge is based on data from (small) retrospective series. Clinical manifestations of hepatic venous obstruction Obstruction of the hepatic veins gives rise to several hemodynamic changes, such as a de- creased sinusoidal blood flow and an increased sinusoidal blood pressure, which can lead to portal hypertension. Venous congestion also provokes ischemia and may subsequently cause necrosis of sinusoidal hepatocytes. Significant hypoxic damage can result in a de- terioration of hepatic synthetic function. Over time, hepatocytes are replaced by fibrosis, predominantly localized in the centrilobular area. Nodular regeneration is also regularly seen in patients with BCS and ultimately, cirrhosis may develop. 6 Other potential consequences of hepatic venous obstruction are portal vein thrombosis and hypertrophy of the caudate lobe. In approximately 15-20 % of cases of BCS concomitant portal vein thrombosis is identified. 7-8 Because the caudate lobe is the only liver segment with direct venous drainage into the inferior vena cava, compensatory hypertrophy often occurs. Caudate hypertrophy itself can subsequently cause compression and stenosis of the inferior vena cava, further contributing to the already existent venous congestion. 9 Clinical presentation of patients with BCS is heterogeneous and ranges from the absence of symptoms to severe liver failure. The classical triad consists of abdominal pain, ascites and hepatomegaly but other possible symptoms are nausea, fever and jaundice. 10 The severity of disease is influenced by the extent of thrombosis, the rapidity of onset and the ensuing effect of compensatory mechanisms such as the formation of collateral veins. In the past years, different classifications (i.e. acute, subacute and chronic) have been used to describe patients with BCS according to the duration and severity of symptoms. 5 However, the prognostic value of these descriptive categories has not been validated. Instead, more recent studies have attempted to determine distinct prognostic classes based on the outcome of clinical and laboratory assessments. 11-12 Despite the major hemodynamic changes involving the liver, synthetic function is often relatively spared. However, this does not preclude a late decline in general condition and liver function. During the course of the disease, portal hypertension frequently develops and may be complicated by bleeding from gastroesophageal varices. In a significant number of pa- tients signs of portal hypertension, such as splenomegaly or esophageal varices, are already present at diagnosis, implicating that an acute thrombotic event can be superimposed on a long-standing obstruction. Less common, an episode of gastrointestinal bleeding is the first presenting sign of BCS. 13-14 In contrast, ascites is an important complication of hepatic venous obstruction and a frequent cause of morbidity. Control of ascites is therefore important in the management of patients with BCS. 12 Introduction Etiology BCS can be further classified as primary or secondary, depending on the underlying cause and the type of venous obstruction. If an endoluminal venous lesion is present, such as thrombosis or an inferior vena cava web, BCS is considered primary. The secondary form con- Chapter 1 sists of venous obstruction caused by external invasion or compression of the venous lumen, as may be caused by malignant tumors or large cysts. 3 Whereas in the past many cases were designated as idiopathic 5, 15, it has nowadays been established that in most patients with BCS an underlying risk factor predisposing to thrombosis is present.
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