Division of Hematology/Oncology, Department of Medicine, Northwestern University Feinberg School of Medicine, 676 N. St. Clair Street, Suite 21-100, Chicago, IL, 60611, USA.
Division of Hematology/Oncology, Department of Medicine, Northwestern University Feinberg School of Medicine, 645 N. Michigan Avenue, Suite 1020, Chicago, IL, 60611, USA.
Curr Hematol Malig Rep. 2018 Jun;13(3):183-190. doi: 10.1007/s11899-018-0446-x.
To review the epidemiology, diagnostic challenges, pathogenesis, and treatment strategies for patients with myeloproliferative neoplasm-associated splanchnic vein thrombosis.
The epidemiology of myeloproliferative neoplasm-associated splanchnic vein thrombosis (MPN-SVT) has been well characterized. While typical MPN-associated thrombosis affects older patients and involves the arterial circulation, MPN-SVT mostly impacts younger women. An association with JAK2 V617F is well-known; recent studies have demonstrated only a weak association with CALR mutations. JAK inhibition may represent a novel treatment strategy, complementing anticoagulation, and management of portal hypertension. While the epidemiology has been well characterized, more work is needed to identify novel contributors to disease pathogenesis, beyond the JAK2 V617F mutation itself, and endothelial compromise. Testing for MPN mutations in the setting of non-cirrhotic SVT is commonplace; JAK2 V617F is the most likely to be identified. Testing for CALR or MPL mutations requires clinical judgement, though not unreasonable. The mainstay of therapy is indefinite anticoagulation; the role of direct oral anticoagulants is unclear. JAK inhibition may play a role in addressing associated splenomegaly and portal hypertension.
综述骨髓增殖性肿瘤相关脾静脉血栓形成（MPN-SVT）的流行病学、诊断挑战、发病机制和治疗策略。
骨髓增殖性肿瘤相关脾静脉血栓形成（MPN-SVT）的流行病学已得到充分描述。虽然典型的 MPN 相关血栓形成影响老年患者且涉及动脉循环，但 MPN-SVT 主要影响年轻女性。JAK2 V617F 的相关性是众所周知的；最近的研究表明，它与 CALR 突变的相关性较弱。JAK 抑制可能代表一种新的治疗策略，补充抗凝和门静脉高压的治疗。尽管流行病学已得到充分描述，但仍需要更多的工作来确定除 JAK2 V617F 突变本身和内皮损伤之外，导致疾病发病机制的新因素。在非肝硬化性 SVT 情况下检测 MPN 突变很常见；最有可能发现 JAK2 V617F。CALR 或 MPL 突变的检测需要临床判断，但并非不合理。治疗的主要方法是无限期抗凝；直接口服抗凝剂的作用尚不清楚。JAK 抑制可能在解决相关的脾肿大和门静脉高压方面发挥作用。
