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Natural history of polycythemia vera and essential thrombocythemia presenting with splanchnic vein thrombosis

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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.

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Acknowledgments

We are indebted to investigators from GEMFIN and REHEVASC for their essential collaboration in data collection.

Funding

This work was supported by the grant PI18/00205 from the Instituto de Salud Carlos III (ISCIII), through the Plan Estatal de Investigación Científica y Técnica y de Innovación. JCGP received support in part by grants from Ministerio de Educación y Ciencia (SAF-2016-75767-R) from ISCCIII (PI17/00398) and from Comissionat per a Universitats i Recerca de la Generalitat de Catalunya (AGAUR SGR 2017). REHEVASC is funded by Asociación Española para el Estudio del Hígado (AEEH). CIBERehd is funded by the Instituto de Salud Carlos III. This work was co-funded by the European Regional Development Fund (ERDF).

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Authors and Affiliations

  1. Hematology Department, Hospital Clínic, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Villarroel 170, 08036, Barcelona, Spain

    Alberto Alvarez-Larrán, Marta Garrote & Francisco Cervantes

  2. Hemotherapy Department, Hospital Clínic, IDIBAPS, Barcelona, Spain

    Arturo Pereira

  3. Barcelona Hepatic Haemodynamic Lab, Liver Unit, Hospital Clínic, IDIBAPS and CIBERehd, Barcelona, Spain

    Marta Magaz, Fanny Turon, Virginia Hernandez-Gea & Juan Carlos García-Pagán

  4. Hematology Department, Hospital Clínico-INCLIVA, Valencia, Spain

    Juan Carlos Hernández-Boluda

  5. Hematology Department, Hospital Universitario de Burgos, Burgos, Spain

    Beatriz Cuevas

  6. Hematology Department, Hospital Morales-Messeguer, Murcia, Spain

    Francisca Ferrer-Marín

  7. Hematology Department, Hospital Dr Negrín, Las Palmas de Gran Canaria, Spain

    M. Teresa Gómez-Casares

  8. Hematology Department, Hospital Ramón y Cajal-IRYCIS, Madrid, Spain

    Valentín García-Gutiérrez

  9. Hematology Department, Hospital Costa del Sol, Marbella, Spain

    M. Isabel Mata-Vázquez

  10. Hemostasis Department, Hospital Clínic, IDIBAPS, Barcelona, Spain

    Eduardo Arellano-Rodrigo

Authors
  1. Alberto Alvarez-Larrán
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  2. Arturo Pereira
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  3. Marta Magaz
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  4. Juan Carlos Hernández-Boluda
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  5. Marta Garrote
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  6. Beatriz Cuevas
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  8. M. Teresa Gómez-Casares
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  9. Valentín García-Gutiérrez
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  10. M. Isabel Mata-Vázquez
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  11. Fanny Turon
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  12. Virginia Hernandez-Gea
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  13. Eduardo Arellano-Rodrigo
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  14. Francisco Cervantes
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  15. Juan Carlos García-Pagán
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Consortia

on behalf of GEMFIN and REHEVASC groups

Contributions

AAL designed the study, collected the data, performed the statistical analysis, analyzed and interpreted the results, and wrote the paper. AP designed the study, performed the statistical analysis, analyzed and interpreted the results, and wrote the paper. MM collected the data, interpreted the results, and wrote the paper. JCHB and FC interpreted the results and wrote the paper. JGP designed the study, interpreted the results, and wrote the paper. MG, BC, FFM, MTGC, VGG, MIMV, FT, and VHG collected the data and approved the final version. A complete list of investigators participating in GEMFIN and REHEVASC studies is provided in supplementary appendix.

Corresponding author

Correspondence to Alberto Alvarez-Larrán.

Ethics declarations

Informed consent for the scientific use of the patients’ clinicohematological data was obtained, and the study was approved by the Hospital Clínic ethics’ committee.

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The authors declare that they have no conflict of interest.

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Alvarez-Larrán, A., Pereira, A., Magaz, M. et al. Natural history of polycythemia vera and essential thrombocythemia presenting with splanchnic vein thrombosis. Ann Hematol 99, 791–798 (2020). https://doi.org/10.1007/s00277-020-03965-z

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  • DOI: https://doi.org/10.1007/s00277-020-03965-z

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