Abstract Number: PB2258
Meeting: ISTH 2020 Congress
Background: Budd Chiari Syndrome (BCS) incidence is 0,5-1 case per million per year. 55-75% are women. There is an underlying disease in the 75% of the cases. Fifty percent of BCS is associated to Philadelphia negative myeloproliferative neoplasms (MPN), being policitemia vera the most frequent one. Forty percent of MPN are JAK2 positive. One third of the BCS are caused by thrombophilia.
The severity of clinical presentation depends on extension and velocity of vein occlusion. Anticoagulation is the main treatment. If clinical manifestations are severe or if there is not response to anticoagulation, it is mandatory to perform endovascular intervention or surgery.
Aims: To describe clinical characteristics, hematological diseases associated and treatment in patients with BCS with more than 5 years of follow up.
Methods: Retrospective review of 6 patients with BCC treated at a university center in Buenos Aires with a follow up of more than 5 years
Results: Six patients were evaluated. Three patients were women (table 1). The median of age at diagnosis was 25 years (IQR 19-30 years) (figure 1). Jak2 positive MPN were present in 5 patients, all of them were treated with hydroxyurea and anticoagulation. Five patients were treated with invasive procedures: 5 transyugular intrahepatic portosystemic shunt (TIPS), 1 angioplasty, 2 thrombus aspirations. None of the patients presented complications during the procedure and all of them are alive. The median of follow up was 11 years (IQR 8-21). In 3 cases, TIPS stenosis developed after a median time of 26 months (IQR 12-74). Angioplasty with TIPS revision and Stent collocation was successfully performed in all these patients.
Conclusions: The combination of anticoagulation and invasive procedures is an interesting option to treat these patients, achieving excellent evolution. It is mandatory to treat the underline disease as well. A multidisciplinary approach is important to guarantee success.
To cite this abstract in AMA style:Mezzarobba D, Schutz N, Penchasky D, Viñuales E, Privitera V, Chuliber F, Villagra Iturre M, Bandi J, Nucifora E. Budd Chiari Syndrome: Beyond 5 Years of Follow up [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/budd-chiari-syndrome-beyond-5-years-of-follow-up/. Accessed January 26, 2022.
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