A review of the therapeutic agents used in the management of polycythaemia vera

Abstract

The acquired clonal disorder Polycythaemia Vera leads to increased erythropoiesis, myelopoiesis and megakaryopoeisis. These anomalies result in an increased incidence of thromboembolic events, transformation to acute leukaemia and myelofibrosis. Treatments which aim to reduce the event rate may increase anaemia but may also affect the rate of complications. This paper reviews the evidence for the treatments which have been used in the management of the disorders over a 50 plus year period. Assessment of this evidence and its limitations form the basis for the current suggested management plans.

A retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs

“…Lowdose acetyl-salicylic acid (ASA) has been recommended too, unless contraindicated, in order to reduce thrombotic risk and mortality; conversely cytoreductive therapy is recommended only in the presence of poor tolerance to phlebotomy, symptomatic or progressive splenomegaly or evidence of high thrombotic risk or disease progression [18,[26][27][28][29].…”

“…For chemotherapy-requiring patients, hydroxyurea (HU) is generally recommended as first-line agent because of its unproven leukemogenicity [28]. However some alkylating agents like pipobroman and busulfan are still used, particularly in elderly patients [26].…”

Simply red: A novel spectrophotometric erythroid proliferation assay as a tool for erythropoiesis and erythrotoxicity studies

“…These drugs currently include hydroxycarbamide (hydroxyurea), pipobroman or interferon, but these therapies can increase the risk of transformation to myelofibrosis or leukemia [21], [26]. An erythroid proliferation assay based on PV hematopoietic stem cells could therefore significantly facilitate the screening for novel compounds that reduce erythroid proliferation to normal levels in this type of myeloproliferative disorder.…”

Nursing Care in Polycythemia Vera: A Review Article

“…Polycythemia vera (PV) is a chronic myeloproliferative neoplasm, which is characterised with the clonal proliferation of the erythroid, myeloid and megakaryocytic series and which is a threat to life [1,2]. The foundation is prepared for the formation of blood clots in all of the tissues and organs, together with an increase in the viscosity and volume of blood in particular, and many systems such as the central nervous system and the cardiovascular and gastrointestinal systems are affected [1,3].…”