Erschienen in:
22.02.2020 | Original Article
Natural history of polycythemia vera and essential thrombocythemia presenting with splanchnic vein thrombosis
verfasst von:
Alberto Alvarez-Larrán, Arturo Pereira, Marta Magaz, Juan Carlos Hernández-Boluda, Marta Garrote, Beatriz Cuevas, Francisca Ferrer-Marín, M. Teresa Gómez-Casares, Valentín García-Gutiérrez, M. Isabel Mata-Vázquez, Fanny Turon, Virginia Hernandez-Gea, Eduardo Arellano-Rodrigo, Francisco Cervantes, Juan Carlos García-Pagán, on behalf of GEMFIN and REHEVASC groups
Erschienen in:
Annals of Hematology
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Ausgabe 4/2020
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Abstract
Patients with polycythemia vera (PV) or essential thrombocythemia (ET) presenting with splanchnic vein thrombosis (SVT) might have a specific clinico-biological profile. To investigate this hypothesis, 3705 PV/ET patients from three national registers, 118 of them presenting with SVT, were reviewed. After correction for age and sex, PV/ET patients with SVT showed an increased risk of death (HR 2.47, 95% CI 1.5–4.01, p < 0.001), venous thrombosis (IRR 3.4, 95%CI 2.1–5.5, p < 0.001), major bleeding (IRR 3.6, 95%CI 2.3–5.5, p < 0.001), and second cancer (IRR 2.37, 95%CI 1.4–4.1, p = 0.002). No case of acute leukemia was documented among patients with PV/ET presenting with SVT and seven of them (6%) progressed to myelofibrosis. SVT was not associated with lower risk of MF after correction by age and sex. Patients with SVT more frequently died from complications related to hepatic disease, major bleeding, or second cancer, resulting in a 5-year reduction of age- and sex-adjusted median survival. In conclusion, PV and ET patients presenting with SVT have shorter survival than patients with PV and ET of the same age and sex. This excess mortality is related to liver disease, major bleeding, and second cancer rather than to the natural evolution of the MPN.