The Royal Free Sheila Sherlock Liver Centre, Royal Free Hospital, Royal Free Hampstead NHS Trust and Institute of Liver and Digestive Health, University College London, Pond Street, NW3 2QG London, United Kingdom.
Best Pract Res Clin Gastroenterol. 2013 Aug;27(4):513-30. doi: 10.1016/j.bpg.2013.06.012.
Prothrombotic haematological disorders, in particular myeloproliferative disorders, are identified in a significant proportion of patients with Budd-Chiari syndrome and portal vein thrombosis (PVT). Multiple prothrombotic disorders may coexist. PVT is diagnosed in one fourth of patients with cirrhosis and is more common with advanced liver disease and hepatocellular carcinoma. PVT in cirrhosis can precipitate decompensation. Intrahepatic microthrombosis may play a role in the pathogenesis of hepatic fibrosis. Sinusoidal obstruction syndrome is usually a complication of myeloablative treatment before haematopoietic stem cell transplantation. Post-transplant lymphoproliferative disorders can complicate liver transplantation and are related to Epstein-Barr virus infection. Hepatitis B reactivation in patients receiving chemotherapy for haematological malignancies is very common without pre-emptive treatment, and can lead to liver failure. Liver involvement is common in primary haematological diseases, such as haemolytic anaemias, lymphomas and leukaemia.
促血栓形成性血液学疾病,特别是骨髓增生性疾病,在相当一部分布加综合征和门静脉血栓形成(PVT)患者中被发现。多种促血栓形成疾病可能同时存在。PVT 在四分之一的肝硬化患者中被诊断出来,并且在晚期肝病和肝细胞癌中更为常见。肝硬化中的 PVT 可导致失代偿。肝内微血栓可能在肝纤维化发病机制中起作用。窦状隙阻塞综合征通常是造血干细胞移植前骨髓清除治疗的并发症。肝移植后的淋巴组织增生性疾病可能使肝移植复杂化,并且与 Epstein-Barr 病毒感染有关。未进行预防性治疗的情况下,接受血液系统恶性肿瘤化疗的患者乙型肝炎病毒再激活非常常见,可能导致肝功能衰竭。原发性血液系统疾病,如溶血性贫血、淋巴瘤和白血病,常伴有肝脏受累。