The treatment of polycythaemia vera: an update in the JAK2 era

Abstract

The clinical course of polycythaemia vera is marked by a high incidence of thrombotic complications, which represent the main cause of morbidity and mortality. Major predictors of vascular events are increasing age and previous thrombosis. Myelosuppressive drugs can reduce the rate of thrombosis, but there is concern that their use raises the risk of transformation into acute leukaemia. To tackle this dilemma, a risk-oriented management strategy is recommended. Low-risk patients should be treated with phlebotomy and low-dose aspirin. Cytotoxic therapy is indicated in high-risk patients, with the drug of choice being hydroxyurea because its leukaemogenicity is low. The recent discovery of JAK2 V617F mutation in the vast majority of polycythaemia vera patients opens new avenues for the treatment of this disease. Novel therapeutic options theoretically devoid of leukaemic risk, such as alpha-interferon and imatinib, affect JAK2 expression in some patients. Nevertheless, these drugs require further clinical experience and, for the time being, should be reserved for selected cases.

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

“…This certainly contributed to the high proportion (70%) of patients in the group of high thrombotic risk. In spite of these unfavorable prognostic features, the overall survival (91% at the median follow-up time of 5.84 years) compares favorably to that reported in many other studies [3][4][5][6]. Although we did not perform a prospective, randomized study and the median follow-up is shorter than 10 years, the wide use in our patients of lowdose ASA and the strong limitation of alkylating cytotoxic therapy may have contributed to this more favorable outcome and to an incidence of leukemic transformation in the lowest range of values reported in literature [4,[6][7][8].…”

“…Polycythemia vera (PV) is the most common chronic myeloproliferative neoplasm, with an annual incidence of 2.3 per 100,000 [1,2], a reported median survival of 10-15 years [3][4][5][6] and an overall mortality of 3.5 per 100 person/year [5]. Thrombotic complications are the main cause of morbidity and mortality, occurring in more than one third of patients and causing 35-45% of deaths [5,6].…”

“…Thrombotic complications are the main cause of morbidity and mortality, occurring in more than one third of patients and causing 35-45% of deaths [5,6]. Other possible adverse events are the evolution into secondary myelofibrosis (MF) or acute myeloid leukemia (AML) occurring in 10-15% [4,[6][7][8] and 2-15% [4,[8][9][10][11][12][13][14][15][16] of patients, respectively, depending on treatments and with increased incidence with long-standing disease.…”

Absence of Hypoalbuminemia Despite Nephrotic Proteinuria in Focal Segmental Glomerulosclerosis Secondary to Polycythemia Vera

“…Secondary FSGS may be the result of hypertrophy and hyperfiltration developing to compensate for various causes, and can also be observed in certain diseases resolving with renal damage (2,3). Polycythemia vera (PV) is a disease characterized by abnormal proliferation in the erythroid series as a result of clonal proliferation of bone marrow progenitor cells (4). Polycythemia may appear in the course of various renal diseases, though the number of case reports of glomerulonephritis developing secondary to PV is limited (5).…”

Nursing Care in Polycythemia Vera: A Review Article

“…Stomach disorders and bleeding may develop in patients due to the use of aspirin, while iron deficiency, exhaustion, symptoms of confusion, nausea-vomiting, difficulty in reaching the peripheral vein, bruising in the needle puncture site following the procedure, hematoma, arm pain and more rarely, anaemia and heart disease may develop in connection with phlebotomy [9,10]. The hydroxyurea used in the treatment of the patients in the medium-high risk group is generally well tolerated by patients, but changes in the skin (loss of hair, hyperpigmentation, erythema, atrophy on the skin, changes in the nails, leg ulcerations) and secondary malignancies do emerge depending on the dose [8,[11][12][13]. IFN-α is mostly recommended in high risk patients below the age of 40, women in the reproductive age group, who have and are unable to use cytoreductive medication due to its teratogenic effects and for those who suffer from severe itching.…”