Riescher-Tuczkiewicz A, Elkrief L, Rautou P-E
Université Paris-Cité, Inserm, centre de recherche sur l'inflammation, UMR 1149, Paris, France.
Université de Tours, service d'hépato-gastro-entérologie, CHRU de Tours, Tours, France.
Rev Med Interne. 2024 Jan;45(1):17-25. doi: 10.1016/j.revmed.2023.07.005. Epub 2023 Oct 12.
Splanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. A rapid and comprehensive workup for thrombosis risk factors is necessary in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis, and is associated with a worse course of cirrhosis. Indications for anticoagulation in patients with cirrhosis are increasing. Transjugular intrahepatic portosystemic shunt is a second-line procedure in this setting. Because of the rarity of these diseases, high-level evidence studies are rare. However, collaborative studies have provided a better understanding of their natural history and allowed to improve the management of these patients. This review focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, patients with portal vein thrombosis without underlying liver disease, and patients with cirrhosis and portal vein thrombosis.
内脏静脉血栓形成包括布加综合征和门静脉血栓形成。这些疾病具有共同特征：（i）它们是罕见病；（ii）它们可导致门静脉高压及其并发症。无潜在肝脏疾病的布加综合征和门静脉血栓形成有许多共同的危险因素，最常见的是骨髓增殖性肿瘤。对这些患者进行快速、全面的血栓形成危险因素检查很有必要。大多数患者需要长期抗凝治疗。门静脉血栓形成也可发生在肝硬化患者中，并与肝硬化病情恶化相关。肝硬化患者抗凝治疗的指征正在增加。经颈静脉肝内门体分流术在此情况下是二线治疗方法。由于这些疾病罕见，高水平证据研究很少。然而，合作研究使人们对其自然病程有了更好的了解，并有助于改善这些患者的管理。本综述重点关注布加综合征患者、无潜在肝脏疾病的门静脉血栓形成患者以及肝硬化合并门静脉血栓形成患者的病因、诊断和管理。
