Is there a gender effect in polycythemia vera?
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REVIEW ARTICLE
Is there a gender effect in polycythemia vera? Francesca Palandri 1 & Barbara Mora 2 & Naseema Gangat 3 & Lucia Catani 1 Received: 3 June 2020 / Accepted: 7 September 2020 # The Author(s) 2020
Abstract In recent times, there has been a growing interest in understanding the impact of gender on disease biology and clinical outcomes in Philadelphia-negative chronic myeloproliferative neoplasms. Among those, polycythemia vera (PV) is characterized by increased thrombotic risk, systemic symptoms, and overall reduced survival. Here, we aim to summarize data on whether and to what extent female sex can affect PV biology and outcome. To this end, we will discuss the latest acquisitions in terms of pathogenesis, diagnosis, epidemiology, clinical presentation and symptoms burden, thrombotic risk and related treatment strategies, and prognosis in female patients affected by PV. Keywords Polycythemia vera . Gender . Therapy . Outcome
Introduction Polycythemia vera (PV) is included among the Philadelphianegative chronic myeloproliferative neoplasms (MPNs), together with essential thrombocythemia (ET) and primary myelofibrosis (PMF) [1]. PV and ET could evolve into post-PV or post-ET myelofibrosis (PPV/PET-MF), also known as secondary myelofibrosis (SMF) [2]. PV is more common than MF [3]. It is characterized by excessive red cell production and the release of pro-inflammatory cytokines [4]. These events are secondary to the hyper-activation of the JAKSTAT pathway, caused by mutations in Janus kinase 2 (JAK2) gene [4]. Clinical phenotype is dominated by systemic symptoms and microvascular disturbances; in 30% of cases, splenomegaly may be detected [5, 6]. In the long term, what really affects outcome is the increased risk of thrombosis, evolution into PPV-MF, or into blast phase (BP) [7]. Gender-based discrepancies have been observed in terms of incidence, response to therapies, and prognosis in several
* Lucia Catani [email protected] Francesca Palandri [email protected] 1
Azienda Ospedaliero-Universitaria di Bologna, Via Albertoni 15, Bologna, Italy
2
Hematology, ASST Sette Laghi, Ospedale di Circolo, Varese, Italy
3
Division of Hematology, Mayo Clinic, Rochester, MN, USA
solid and hematological cancers [8]. Sex differences may depend on multiple factors, including differentially activated genetic/molecular patterns, immune system function, sex hormones expression [9], and drug metabolism [10]. In solid cancers, as in acute leukemia, incidence, and mortality rates are higher in males. Overall mortality depends not only on multiple factors, including cardiovascular risk factors (which are generally more frequent in males), but also on genetic polymorphisms involved in facilitating carcinogenesis [11]. Besides, increased predisposition to leukemia in males could depend on the lack of estrogens [12]. The latter are supposed to inhibit the Nuclear Factor kappa B (NF-κB) pathway, which regulates the transcription of Interferon Regulatory Factor 4 (IRF4) and, as a consequence, a correct differe
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