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Thrombosis in polycythemia rubra vera

  • 07
    By Hratch L Karamanoukian MD FACS RVT RPVI RPhS DABVLM (2009)

    I recently saw a young female patient who was diagnosed with polycythemia and she came to see me because of concern for developing thromboembolism.

    I forwarded her this nice article and I am summarizing the results for the readers.

    The study was published by Martin Griesshammer and colleagies from University Clinic for Hematology, Oncology, Hemostaseology and Palliative Care, Johannes Wesling Medical Center Minden, UKRUB, University of Bochum, Hans-Nolte-Straße 1, 32429, Minden, Germany; Hôpital Saint-Louis, AP-HP, Centre d'Investigations Cliniques (CIC 1427), Université Paris Diderot, INSERM UMRS 1131, 1 Avenue Claude Vellefaux, Paris, France; and 
    Hospital del Mar-IMIM, Passeig Marítim 25-29, 08003, Barcelona, Spain

    Compared to other myeloproliferative disorders. the authors state that thromboembolic events and cardiovascular disease are the most prevalent complications in patients with polycythemia vera (PV).

    Furthermore, the major cause of morbidity and mortality in patients with PV.

    The authors state that the most likley scenario for clinicans to make the diagnosis of PV is after a vascular complication such as arterial or venous thrombosis.

    The highest rates of thrombosis typically occur shortly before or at diagnosis and decrease over time.

    What are risk factors for the development of thromboembolic complications in PV? 

    age (≥ 60 years old) and a history of thrombosis

    elevated hematocrit


    What are the goals of therapy for PV ? 

    controlling hematocrit to < 45%, a target associated with reduced rates of cardiovascular death and major thrombosis.

    How are patients with PV managed ? 

    Low-risk patients (< 60 years old with no history of thrombosis) are managed with phlebotomy and low-dose aspirin

    high-risk patients (≥ 60 years old and/or with a history of thrombosis) should be treated with cytoreductive agents.

    Interferon and ruxolitinib are considered second-line therapies for patients who are intolerant of or have an inadequate response to hydroxyurea, which is typically used as first-line therapy.

    Reference:  Greisshammer M, Kiladjian JJ, Besses C.  Thromboembolic events in polycythemia. Ann Hematol2019; 98: 1071-1082.