Patients with myeloproliferative neoplasms (MPNs) who develop portal hypertension (pHTN) will likely require life-long anticoagulation to manage the condition. These findings were published in Blood Coagulation & Fibrinolysis.

pHTN is a common occurrence in patients with MPNs (7% to 18%); however, there is a paucity of data about the efficacy of direct oral anticoagulants (DOACs) after transjugular intrahepatic portosystemic shunt (TIPS). Researchers from the University of Utah discussed a case series of 2 patients with MPN-associated pHTN to assess the evidence for treatment efficacy.

Patient 1 was a 23-year-old woman who presented with persistent headache 6 months after receiving a diagnosis of Budd-Chiari syndrome. She experienced heavy menstrual bleeding and was switched from warfarin to rivaroxaban therapy. At 6 weeks, she discontinued rivaroxaban due to persistent bleeding. After receiving low molecular weight heparin (LMWH), her headaches and menstrual bleeding resolved. She later underwent a TIPS procedure, and 2 months after, required recanalization and venoplasty due to an occluded TIPS stent. At 1 year after TIPS, she presented with TIPS re-thrombosis and underwent thrombectomy of the portal vein and TIPS stent with a balloon angioplasty. She was discharged with LMWH.

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Patient 2 was a 57-year-old woman with MPN. She experienced lower abdominal pain due to extensive thromboses and was given apixaban. At 3 months, she underwent a portal vein reconstruction-TIPS procedure. At 11 months, she presented with TIPS stent occlusion and required TIPS revision with thrombectomy and venoplasty; she was continued on apixaban. Six months later, she was found to have TIPS stent re-occlusion and underwent an angioplasty. She was switched to LMWH.

These cases suggest that TIPS failure may be common among patients with MPN-associated pHTN. Due to lack of efficacy, DOACs should not be considered as standard of care.


Abou-Ismail MY, Prchal JT, Deininger MW, Parker CJ, Lim MY. Anticoagulation management post-transjugular intrahepatic portosystemic shunt in portal hypertension associated with myeloproliferative neoplasms. Blood Coagul Fibrinolysis. Published online October 4, 2021. doi:10.1097/MBC.0000000000001087