Management of polycythemia vera: A survey of treatment patterns in Italy

Eur J Haematol. 2023 Feb;110(2):161-167. doi: 10.1111/ejh.13889. Epub 2022 Nov 10.

Abstract

Objectives: Polycythemia vera (PV) is an acquired clonal hematopoietic stem cell disorder characterized by the overproduction of red blood cells. It has long been underlined that there are differences in treatment patterns in routine practice. Therapeutic strategies have also expanded, and in recent years the JAK1/JAK2 inhibitor ruxolitinib has emerged as a second-line therapeutic option in patients who are intolerant to or resistant to hydroxyurea. Determining the impact of changes on practice patterns is of interest, especially for aspects that lack detailed guidance for management.

Methods: To gain insights into treatment patterns by clinicians treating patients with PV in Italy, we carried out a survey of 60 hematologists and transfusion specialists. The questions covered: treatment of low-risk patients, definition of significant leukocytosis, splenomegaly and excessive phlebotomies, resistance/intolerance to hydroxyurea, use of ruxolitinib, cytoreductive therapy, and vaccines.

Results: In general, the results of the survey indicate that there is a large heterogeneity in management of patients with PV across these areas.

Conclusions: While helping to provide greater understanding of treatment patterns for patients with PV in Italy, our survey highlights the need for additional clinical studies to obtain more precise guidance for the routine care of patients with PV.

Keywords: management; patterns; polycythemia vera; survey; treatment.

MeSH terms

  • Humans
  • Hydroxyurea / therapeutic use
  • Italy / epidemiology
  • Janus Kinase 2
  • Janus Kinase Inhibitors* / therapeutic use
  • Polycythemia Vera* / diagnosis
  • Polycythemia Vera* / epidemiology
  • Polycythemia Vera* / therapy
  • Pyrazoles / therapeutic use

Substances

  • Hydroxyurea
  • ruxolitinib
  • Pyrazoles
  • Janus Kinase 2
  • Janus Kinase Inhibitors

Grants and funding