ABSTRACT
En la cirrosis hepática es frecuente que se produzca activación de la trombina y microtrombosis en las raicillas de la vena porta intrahepática, en parte debido al déficit de proteína C, y en parte a alteración del equilibrio coagulación-anticoagulación-fibrinólisis. Por eso hay una incidencia aumentada de trombosis portal. La trombina no solo puede generar la formación de un trombo, sino que puede activar a las células estrelladas y estimular la fibrogénesis. Además, la isquemia asociada a la trombosis puede promover la síntesis de factores de crecimiento involucrados en la fibrogénesis. La coincidencia en un mismo paciente de mutaciones protrombóticas, como factor V Leiden o polimorfismos del PAI- 1, puede acelerar todo este proceso. Presentamos dos casos de cirrosis criptogenética en los que los únicos factores identificables capaces de causar fibrogénesis acelerada fueron mutaciones del factor V y del PAI-1. Estas observaciones, además de apoyar la hipótesis de que las citadas mutaciones puedan por sí mismas llegar a provocar cirrosis, sugieren que es recomendable determinar si existen polimorfismos del factor V, PAI-1 y protrombina en el estudio de la cirrosis criptogenética (AU)
Thrombin activation and microthrombosis of intrahepatic portal venules is a common feature in liver cirrhosis, due in part to relative protein C deficiency and altered coagulation-anticoagulation-fibrinolysis balance. Extension of this microthrombotic process to larger portal vessels explains the increased incidence of portal vein thrombosis in liver cirrhosis. Thrombin not only leads to thrombosis, but also activates liver stellate cells and promotes fibrogenesis. Also, ischemia associated with thrombosis up-regulates the expression and secretion of growth factors involved in fibrogenesis. The coincidence in a given patient of prothrombotic mutations, such as factor V Leiden or PAI-1 polymorphisms, can accelerate the fibrogenetic process. We hereby present two cases of liver cirrhosis in which etiologic evaluation was negative except for the finding of a factor V Leiden mutation in one case and the 4G/5G PAI polymorphism in the second case. These observations support the hypothesis that these mutations may be involved in the etiology of some cases of cirrhosis, or, at least, accelerate the evolution of the disease. It is therefore convenient to search for the presence of prothrombotic mutations in patients with cryptogenetic cirrhosis(AU)
Subject(s)
Humans , Female , Middle Aged , Aged , Liver Cirrhosis/complications , Factor V/genetics , Portal Vein , Venous Thrombosis/genetics , Risk Factors , Genetic Predisposition to Disease , C-Reactive Protein/analysisABSTRACT
A 41-year-old woman was admitted to the emergency room with symptoms compatible with deep vein thrombosis affecting the left lower extremity and pulmonary thromboembolism. A CT scan was consistent with pulmonary emboli and thrombosis of the iliac veins extending to the inferior vena cava, which persisted even after correct systemic fibrinolytic therapy. For this reason, a venography was performed and local thrombolysis was administered. Venography revealed a compression of the left common iliac vein caused by the right common iliac artery, so that the patient was diagnosed with May-Thurner syndrome. The clinical features of this anatomical condition and sometimes lethal clinical syndrome are discussed
No disponible
Subject(s)
Humans , Female , Adult , May-Thurner Syndrome/complications , Pulmonary Embolism/etiology , Venous Thrombosis/physiopathology , Contraceptives, Oral/adverse effects , Venous Insufficiency/complications , Risk FactorsABSTRACT
Thrombin activation and microthrombosis of intrahepatic portal venules is a common feature in liver cirrhosis, due in part to relative protein C deficiency and altered coagulation-anticoagulation-fibrinolysis balance. Extension of this microthrombotic process to larger portal vessels explains the increased incidence of portal vein thrombosis in liver cirrhosis. Thrombin not only leads to thrombosis, but also activates liver stellate cells and promotes fibrogenesis. Also, ischemia associated with thrombosis up-regulates the expression and secretion of growth factors involved in fibrogenesis. The coincidence in a given patient of prothrombotic mutations, such as factor V Leiden or PAI-1 polymorphisms, can accelerate the fibrogenetic process. We hereby present two cases of liver cirrhosis in which etiologic evaluation was negative except for the finding of a factor V Leiden mutation in one case and the 4G/5G PAI polymorphism in the second case. These observations support the hypothesis that these mutations may be involved in the etiology of some cases of cirrhosis, or, at least, accelerate the evolution of the disease. It is therefore convenient to search for the presence of prothrombotic mutations in patients with cryptogenetic cirrhosis.
