How I treat polycythemia vera

Abstract

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm associated with JAK2 mutations (V617F or exon 12) in almost all cases. The World Health Organization has defined the criteria for diagnosis, but it is still unclear which parameter (hemoglobin or hematocrit) is the most reliable for demonstrating increased red cell volume and for monitoring response to therapy; also, the role of bone marrow biopsy is being revisited. PV is associated with reduced survival because of cardiovascular complications and progression to post-PV myelofibrosis or leukemia. Criteria for risk-adapted treatment rely on the likelihood of thrombosis. Controlled trials have demonstrated that incidence of cardiovascular events is reduced by sustained control of hematocrit with phlebotomies (low-risk patients) and/or cytotoxic agents (high-risk patients) and antiplatelet therapy with aspirin. Hydroxyurea and interferon may be used as first-line treatments, whereas busulfan is reserved for patients that are refractory or resistant to first-line agents. However, there is no evidence that therapy improves survival, and the significance of reduction of JAK2 mutated allele burden produced by interferon is unknown. PV is also associated with a plethora of symptoms that are poorly controlled by conventional therapy. This article summarizes my approach to the management of PV in daily clinical practice.

The efficacy and safety of continued hydroxycarbamide therapy versus switching to ruxolitinib in patients with polycythaemia vera: a randomized, double‐blind, double‐dummy, symptom study (RELIEF)

“…Polycythaemia vera (PV) is a Philadelphia‐negative myeloproliferative neoplasm (MPN) characterized by primary erythrocytosis and deregulated Janus kinase/signal transducer and activator of transcription signalling (Vannucchi, 2014). Most patients with PV experience a broad symptom burden that may include fatigue/tiredness, itching, muscle aches and sweating (Emanuel et al , 2012; Vannucchi et al , 2015).…”

“…The most common cytoreductive treatment is hydroxycarbamide (HC, also termed hydroxyurea) (Vannucchi, 2014), which is effective for controlling blood cell counts in some patients (Najean & Rain, 1997; Alvarez‐Larran et al , 2012). However, PV‐related symptoms are generally not well controlled with HC treatment (Johansson et al , 2012; Scherber et al , 2012).…”

Policitemia vera: associação entre enfartes cerebrais e úlceras gástricas

“…2,3 No presente caso, os níveis quase indetectáveis de eritrocitose possibilitaram a exclusão de causas secundárias. Por outro lado, foram cumpridos os critérios da OMS necessários para o diagnóstico de PV (ver Tabela 2), nomeadamente os dois critérios major e dois critérios minor.…”

“…1 Todavia, existem sintomas como astenia, prurido (tipicamente aquagénico), cefaleias, parestesias, zumbidos, visão turva, artralgias, desconforto abdominal ou hipersudorese que podem ser atribuídos à eritrocitose, por oposição à tríada de cianose crónica, esplenomegalia e policitemia que foi atribuída à PV ainda no século XIX por Vaquez. 3,4 Existem também associações menos frequentes como a doença ulcerosa péptica que devem ser tidas em conta na PV e podem alertar para o seu diagnóstico.…”

All we know about polycythemia vera: literature review and own experience

“…Наиболее часто встречающиеся отличительные признаки вторичного эритроцитоза и ИП представлены в табл. 3 [50]. Определенную по-мощь в проведении дифференциальной диагностики между истинной полицитемией и вторичными эритро-цитозами может оказать алгоритм проведения диффе-ренциального диагноза, представленный на рис.…”