文章：

骨髓增殖性肿瘤中的内脏静脉血栓形成：181例患者队列中复发的危险因素

Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients

原文发布日期：2016-11-04

DOI: 10.1038/bcj.2016.103

类型: Original Article

开放获取: 是

英文摘要：

We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd–Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.

摘要翻译： 

我们回顾性研究了181例首次发生内脏静脉血栓的真性红细胞增多症（67例）、原发性血小板增多症（67例）或原发性骨髓纤维化（47例）患者。其中31例（17.1%）诊断为布加综合征，109例（60.3%）诊断为门静脉血栓；分别有18例和23例检测到单纯性肠系膜静脉或脾静脉血栓。指数事件后，患者随访总时长为735患者·年，期间发生31例复发事件，相当于每100患者·年4.2例的复发率。与复发风险显著相关的因素包括布加综合征（风险比3.03）、既往血栓病史（风险比3.62）、脾肿大（风险比2.66）和白细胞增多（风险比2.8）。85%的患者接受维生素K拮抗剂治疗，其复发率为每100患者·年3.9例，而未接受维生素K拮抗剂的少数患者（15%）复发率更高（每100患者·年7.2例）。颅内和颅外大出血主要发生在接受维生素K拮抗剂治疗的患者中，发生率为每100患者·年2.0例。结论显示，尽管接受抗凝治疗，骨髓增殖性肿瘤患者发生内脏静脉血栓后的复发率仍较高，建议探索采用新型抗血栓药物和JAK-2抑制剂进行二级预防的新途径。

原文链接：

Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients