Multicenter analysis of the use of transjugular intrahepatic portosystemic shunt for management of MPN‐associated portal hypertension

Abstract

BCR-ABL1-negative myeloproliferative neoplasms (MPNs) are clonal stem cell disorders defined by proliferation of one or more myeloid lineages, and carry an increased risk of vascular events and progression to myelofibrosis and leukemia. Portal hypertension (pHTN) occurs in 7–18% of MPN patients via both thrombotic and nonthrombotic mechanisms and portends a poor prognosis. Transjugular intrahepatic portosystemic shunt (TIPS) has been used in the management of MPN-associated pHTN; however, data on long-term outcomes of TIPS in this setting is limited and the optimal management of medically refractory MPN-associated pHTN is not known. In order to assess the efficacy and long-term outcomes of TIPS in MPN-associated pHTN, we performed a retrospective analysis of 29 MPN patients who underwent TIPS at three academic medical centers between 1997 and 2016. The majority of patients experienced complete clinical resolution of pHTN and its clinical sequelae following TIPS. One, two, three, and four-year overall survival post-TIPS was 96.4%, 92.3%, 84.6%, and 71.4%, respectively. However, despite therapeutic anticoagulation, in-stent thrombosis occurred in 31.0% of patients after TIPS, necessitating additional interventions. In conclusion, TIPS can be an effective intervention for MPN-associated pHTN regardless of etiology. However, TIPS thrombosis is a frequent complication in the MPN population and indefinite anticoagulation post-TIPS should be considered.

Transjugular Intrahepatic Portosystemic Shunt Placement in Patients with Schistosomiasis-Induced Liver Fibrosis

“…Laboratory tests mainly included liver, kidney and coagulation function. The study outcomes were the incidence of hepatic encephalopathy (HE) [24], variceal rebleeding, survival as well as shunt dysfunction which was defined as shunt thrombosis or stenosis resulting in clinical symptoms and/or requiring procedural intervention [25].…”

From Budd-Chiari syndrome to acquired von Willebrand syndrome: thrombosis and bleeding complications in the myeloproliferative neoplasms

“…52 In a retrospective study of 29 patients with MPN, TIPS was effective in resolving pHTN and its consequences, but 31% experienced in-stent thrombosis requiring additional interventions. 65 Finally, in patients with BCS with progressive liver dysfunction, transplantation may be required. 52 Among 78 patients with BCS who underwent liver transplantation, 41 had an MPN (40 with ET or PV) and had long-term survival comparable to that of those without MPN.…”

Characteristics of myeloproliferative neoplasm-associated portal hypertension and endoscopic management of variceal bleeding

“… 7 Previous studies have shown that JAK2 V617 F has a prominent role in vascular risk and MPN-associated PHT. 8 , 9 The mechanisms underlying PHT in MPNs remain unclear. Splanchnic vein thrombosis (SVT) is a studied contributing force and extramedullary hematopoiesis within the liver and spleen also plays a pivotal role.…”