Patient-Centered Focused Review| Volume 365, ISSUE 5, P457-461, May 2023

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Portal vein thrombosis as the presenting manifestation of JAK2 positive myeloproliferative neoplasm

Published:January 09, 2023DOI:


      Deep venous thrombosis (DVT) is a complication of myeloproliferative neoplasms (MPNs). However, DVTs in unusual sites such as portal vein thrombosis (PVT) are rare and may be the first clinical manifestation of occult MPNs. There is a need for increasing awareness of such manifestations; so, here we discuss a patient who presented with new portal vein thrombosis, underwent further studies, was ultimately diagnosed with JAK2 positive MPN, and started on appropriate treatment with improvement of thrombosis and controlled hematocrit.

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        • Arber DA
        • Orazi A
        • Hasserjian R
        • et al.
        The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.
        Blood. 2016; 127: 2391-2405
        • Arachchillage DR
        • Laffan M.
        Pathogenesis and management of thrombotic disease in myeloproliferative neoplasms.
        Semin Thromb Hemost. 2019; 45: 604-611
        • Myers TJ
        • Steinberg WM
        • Rickles FR.
        Polycythemia vera and mesenteric arterial thrombosis. A disease association resulting from decreased platelet sensitivity to aspirin.
        Arch Intern Med. 1979; 139: 695-698
        • Smalberg JH
        • Arends LR
        • Valla DC
        • et al.
        Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis.
        Blood. 2012; 120: 4921-4928
        • Rungjirajittranon T
        • Owattanapanich W
        • Ungprasert P
        • et al.
        A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms.
        BMC Cancer. 2019; 19 (Published 2019 Feb 28): 184
        • Finazzi G
        • De Stefano V
        • Barbui T.
        Splanchnic vein thrombosis in myeloproliferative neoplasms: treatment algorithm 2018.
        Blood Cancer J. 2018; 8 (Published 2018 Jun 26): 64
        • Chim CS
        • Kwong YL
        • Lie AK
        • et al.
        Long-term outcome of 231 patients with essential thrombocythemia: prognostic factors for thrombosis, bleeding, myelofibrosis, and leukemia.
        Arch Intern Med. 2005; 165: 2651-2658
        • Barbui T
        • Carobbio A
        • Cervantes F
        • et al.
        Thrombosis in primary myelofibrosis: incidence and risk factors.
        Blood. 2010; 115: 778-782
        • Elliott MA
        • Pardanani A
        • Lasho TL
        • et al.
        Thrombosis in myelofibrosis: prior thrombosis is the only predictive factor and most venous events are provoked.
        Haematologica. 2010; 95: 1788-1791
        • Enblom A
        • Lindskog E
        • Hasselbalch H
        • et al.
        High rate of abnormal blood values and vascular complications before diagnosis of myeloproliferative neoplasms.
        Eur J Intern Med. 2015; 26: 344-347
        • Duangnapasatit B
        • Rattarittamrong E
        • Rattanathammethee T
        • et al.
        Clinical manifestations and risk factors for complications of philadelphia chromosome-negative myeloproliferative neoplasms.
        Asian Pac J Cancer Prev. 2015; 16: 5013-5018
        • Kaifie A
        • Kirschner M
        • Wolf D
        • et al.
        Bleeding, thrombosis, and anticoagulation in myeloproliferative neoplasms (MPN): analysis from the German SAL-MPN-registry.
        J Hematol Oncol. 2016; 9 (Published 2016 Mar 5): 18
        • Hoekstra J
        • Bresser EL
        • Smalberg JH
        • et al.
        Long-term follow-up of patients with portal vein thrombosis and myeloproliferative neoplasms.
        J Thromb Haemost. 2011; 9: 2208-2214
        • Loscocco GG
        • Guglielmelli P
        • Vannucchi AM.
        Impact of mutational profile on the management of myeloproliferative neoplasms: a short review of the emerging data.
        Onco Targets Ther. 2020; 13 (Published 2020 Dec 1): 12367-12382
        • Spivak JL.
        Myeloproliferative neoplasms.
        N Engl J Med. 2017; 376: 2168-2181
        • Barbui T
        • Finazzi G
        • Falanga A.
        Myeloproliferative neoplasms and thrombosis.
        Blood. 2013; 122: 2176-2184
        • Spivak JL
        • Moliterno AR.
        The thrombopoietin receptor, MPL, is a therapeutic target of opportunity in the MPN.
        Front Oncol. 2021; 11 (Published 2021 Mar 10)641613
        • Tefferi A
        • Barbui T.
        Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
        Am J Hematol. 2020; 95: 1599-1613
        • Foucar CE
        • Stein BL.
        JAK2 V617F Mutation testing in patients presenting with hepatic and portal vein thrombosis.
        JAMA. 2017; 317: 2228-2229
        • Roberts JA
        • Morales M
        • Rice L.
        Progressive anemia and left upper quadrant pain in a patient with polycythemia vera.
        JAMA. 2014; 311: 2227-2228
        • Baumeister J
        • Chatain N
        • Sofias AM
        • et al.
        Progression of myeloproliferative neoplasms (MPN): diagnostic and therapeutic perspectives.
        Cells. 2021; 10 (Published 2021 Dec 16): 3551