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Budd-Chiari Syndrome and Portal Vein Thrombosis Budd-Chiari Syndrome and Portal Vein Thrombosis Etiology and Treatment Jasper H. Smalberg Financial support by the Dutch Heart Foundation for the publication of this thesis is gratefully acknowledged. Publication of this thesis was financially supported by: Fonds Wetenschappelijk Onderzoek van de MPN Stichting, Alexion, Bayer, Celgene, Novartis, Pfizer, Shire, The J.E. Jurriaanse Stichting. Cover: Veins of the hepatic portal system, Science Photo Library Lay-out: Legatron Electronic Publishing Printing: Ipskamp Drukkers BV, Enschede ISBN/EAN: 978-94-6191-144-5 2012 ©J.H. Smalberg No part of this thesis may be reproduced, stored in a retrieval system or transmitted in any form or by any means, without written permission of the author or, when appropriate, of the publishers of the publications. Budd-Chiari Syndrome and Portal Vein Thrombosis Etiology and Treatment Etiologie en behandeling van het 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 donderdag 12 januari 2012 om 09.30 uur door Jasper Hoite Smalberg geboren op 10 juli 1980 te Hellevoetsluis PROMOTIECOMMISSIE Promotor: Prof.dr. F.W.G. Leebeek Prof.dr. H.L.A. Janssen Overige leden: Prof.dr. H.J. Metselaar Prof.dr. R.J. Porte Prof.dr. P. Sonneveld CONTENTS Chapter 1 General introduction and outline of thesis 7 Chapter 2 Hypercoagulability and hypofibrinolysis and risk of deep vein 15 thrombosis and splanchnic vein thrombosis: similarities and differences Chapter 3 Myeloproliferative neoplasms in the pathogenesis and survival 35 of Budd-Chiari syndrome Chapter 4 Myeloproliferative neoplasms in Budd-Chiari syndrome and 53 portal vein thrombosis: a meta-analysis Chapter 5 The JAK2 46/1 haplotype in Budd-Chiari syndrome and portal 73 vein thrombosis Chapter 6 Fibrinogen gamma prime and variation in fibrinogen gamma 89 prime genes in the etiology of portal vein thrombosis Chapter 7 Fibrinogen gamma prime appears to be unrelated to the 103 development of the Budd-Chiari syndrome Chapter 8 Risks and benefits of transcatheter thrombolytic therapy in 109 patients with splanchnic venous thrombosis Chapter 9 Long-term follow-up of patients with portal vein thrombosis 121 and myeloproliferative neoplasms Chapter 10 General discussion 137 Summary 151 Samenvatting 155 Dankwoord 159 List of publications 162 Curriculum vitae 163 PhD portfolio 165 CHAPTER 1 GENERAL INTRODUCTION AND OUTLINE OF THESIS 8 | Chapter 1 Venous thrombosis is a common disorder with an annual incidence of around 1-2 cases per 1.000 individuals and is the third leading cause of cardiovascular morbidity and mortality in developed countries.1-4 Thrombosis may arise in any section of the venous system, but it typically occurs in the deep veins of the lower extremities. The major concern in these patients is pulmonary embolism, which can be fatal. A more common, but often disabling, complication of deep vein thrombosis and its sequelae is the post-thrombotic syndrome.5 Rarely, thrombosis may involve other venous sites. One of these uncommon manifestations of thrombosis is located in the splanchnic veins, which is accompanied by a considerable morbidity and mortality. SPLANCHNIC VEIN THROMBOSIS Splanchnic vein thrombosis (SVT) encompasses hepatic vein thrombosis (Budd-Chiari syndrome, BCS), portal vein thrombosis (PVT), and mesenteric vein thrombosis. BCS and PVT are the two most frequent manifestations of SVT, and although in these disorders distinct venous sites are affected, simultaneous involvement of these venous districts is frequently encountered.6 The splanchnic venous system comprises the portal vein and its branches that direct blood flow from the gastrointestinal organs to the liver. The portal vein is formed by the union of the superior mesenteric vein and the splenic vein, and subdivides in a left and right branch, which are segmentally distributed throughout the liver. The terminal portal venules drain into the sinusoids, after which the blood flows from the small to large hepatic veins, ultimately reaching the inferior vena cava. BCS is defined as an obstruction of the hepatic venous outflow tract from the level of the small hepatic veins to the entrance of the inferior vena cava into the right atrium. Outflow obstruction caused by hepatic veno-occlusive disease and cardiac disorders is excluded from this definition. BCS is considered primary when obstruction of the venous tract is the result of an endoluminal lesion, i.e. thrombosis, and secondary when obstruction results from invasion by a local malignant tumor or from extrinsic compression by a tumor, cyst or abscess.7 BCS is a rare disorder with an annual incidence of about 0.2-0.8 per million inhabitants in the Western world, predominantly affecting young females.8-10 Main complications are the result of portal hypertension and liver dysfunction. The classical triad of symptoms in BCS consists of abdominal pain, ascites and hepatomegaly, frequently accompanied by a variable degree of alterations in liver biochemical tests. However, clinical presentation may range from absence of symptoms, in case of preservation of hepatic veins and/or formation of collaterals, to fulminant hepatic failure, with an acute or chronic development of symptoms ranging from weeks to months.6 With contemporary management, the survival rate is 87% at one year and 82% at two years.11 Introduction | 9 In PVT, the obstruction is located in the extra-hepatic portal vein, but involvement of the intra- hepatic portal, superior mesenteric and splenic vein may occur. Although PVT is considered a rare disorder, a recent autopsy study reported a prevalence of 1%.12 Clinically, PVT can be classified as acute or chronic, which represent successive stages of the same disease 1 Chapter and share similar causes. Complications of portal hypertension, such as gastrointestinal bleeding from oesophageal varices and splenomegaly, are the most important clinical manifestations of PVT.6 Furthermore, if thrombosis extends into the mesenteric vein, there is a substantial risk of bowel infarction, which is the most severe complication of acute PVT.13 The prognosis of patients with PVT is mainly determined by the underlying cause. Survival of patients with non-cirrhotic, non-malignant PVT can be considered good. In this group of PVT patients, five- and ten-year survival rates are 90% and 80%, respectively.14 PVT patients with an underlying liver cirrhosis or hepato-biliary malignancy generally have an inferior prognosis. Etiology Local risk factors for the development of BCS include solid malignancies, parasitic masses, cysts or abscesses that either compress or invade the venous tract.7 In the Western world, BCS is infrequently caused by local risk factors. PVT, on the other hand, is most often encountered as a complication of liver cirrhosis or hepatobiliary malignancies. Other frequent local risk factors for PVT are surgical trauma to the portal vein and inflammatory foci in the abdomen, which are often accompanied by an additional prothrombotic condition. The etiology of primary BCS and non-malignant, non-cirrhotic PVT often involves systemic, prothrombotic conditions. Recent studies with a near complete work-up showed that prothrombotic factors are present in up to 84% and 42% in primary BCS and non- malignant, non-cirrhotic PVT, respectively.11,15 These conditions largely overlap with the risk factors for common venous thrombosis and can be divided into genetic and acquired risk factors. Genetic risk factors include protein C, protein S and antithrombin deficiencies, the factor V Leiden mutation and the prothrombin G20210A gene variant. Acquired risk factors include antiphospholipid antibodies, paroxysmal nocturnal hemoglubinuria, hormonal factors, auto-immune diseases and myeloproliferative neoplasms (MPNs), which remarkably are the most prominent risk factor for the development of both BCS and PVT. MPNs are chronic clonal hematopoietic stem cell disorders characterized by an overproduction of mature and functional granulocytes, red blood cells and/or platelets.16 The exact pathogenetic mechanism of thrombosis in MPNs remains elusive, but besides characteristic erythrocytosis and thrombocytosis, platelet and leukocyte functional abnormalities appear critical.17 MPNs have been reported in approximately one third and one half of BCS and PVT patients, respectively.18 Diagnosis of MPNs in these patients is notoriously difficult. Portal hypertension, resulting from pre- or post-hepatic venous 10 | Chapter 1 obstruction, can lead to hypersplenism and hemodilution. Both these conditions may mask the characteristic peripheral blood cell changes and make diagnosis of MPN more difficult. Previously, diagnosis of MPNs in these patients often relied on bone marrow (BM) biopsy findings and growth of erythroid colonies in the absence of exogenous erythropoietin, referred to as spontaneous endogenous erythroid colonies (EEC). Patients were labelled as having so-called occult MPN when either bone marrow biopsy was highly suggestive of MPN or EEC was present, but in whom traditional criteria for MPN could not be fulfilled due to normal peripheral blood cell counts.19 In 2005, the JAK2V617F gain of function mutation was discovered, which is present in more than 95% of cases of polycythemia vera and 50% to 60% of essential thrombocythemia and primary myelofibrosis. JAK2V617F
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