Abstract
Treatment modalities for polycythemia vera (PV) have evolved over time. Phlebotomy and low-dose aspirin suffice in low-risk patients, but cytoreductive therapies are indicated in all high-risk patients (age ≥ 65 years or those with a history of PV-related thrombotic event) and may be considered for low-risk patients with progressively increasing splenomegaly, progressively increasing leucocyte and platelet counts, and for those who do not tolerate phlebotomy. Hydroxyurea/hydroxycarbamide or interferons can be used as first-line drugs. Hydroxyurea may not be tolerated by some patients, and it also carries risk of myelosuppression. Interferon alfa is especially useful for PV symptoms, and the newer preparation, ropeginterferon alfa-2b, has lesser incidence of flu-like reactions. Ruxolitinib reduces the JAK2V617F mutation burden and is used as a second-line drug. Anagrelide reduces platelet production and can be used in conjunction with hydroxyurea in patients with excessive thrombocytosis. The alkylating agent, busulfan, can also be used as a last resort in patients with a limited life expectancy. Prospective future treatments include givinostat, a histone deacetylase inhibitor, and idasanutlin, a murine double minute 2 antagonist.
Highlights
BackgroundPolycythemia vera (PV), one of the myeloproliferative malignancies, is characterized by clonal proliferation of hematopoietic cells, erythrocytes
A majority of the cases of polycythemia vera (PV) are accompanied by Janus kinase-2 (JAK-2) mutations, mainly the JAK2V617F mutation [1]
This results in a nucleotide switch and, in turn, a qualitative genetic abnormality resulting in replacement of valine by phenylalanine at codon 617 on chromosome 9 [2]. The identification of this mutation has led to the discovery of novel treatment options, which have revolutionized the management of PV
Summary
Polycythemia vera (PV), one of the myeloproliferative malignancies, is characterized by clonal proliferation of hematopoietic cells, erythrocytes. A majority of the cases of PV are accompanied by Janus kinase-2 (JAK-2) mutations, mainly the JAK2V617F mutation [1] This results in a nucleotide switch and, in turn, a qualitative genetic abnormality resulting in replacement of valine by phenylalanine at codon 617 on chromosome 9 [2]. The clinical spectrum of PV is vast from being detected incidentally to a full blown picture with typical signs and symptoms pertaining to increased red blood cell (RBC) number and mass, thereby leading to increased blood viscosity. This predisposes to thrombotic complications along with hemorrhagic complications due to production of dysfunctional platelets. The cornerstone of management includes reducing thrombotic events, managing constitutional symptoms, and halting the progression of malignancy while minimizing side effects associated with various therapeutic modalities [4]
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