Transformation of polycythemia vera into myeloproliverative neoplasm accelerated phase and postpolycythemia vera myelofibrosis in a 67-year-old male

Abstract

Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) characterized by increased blood cells, especially red blood cells. The most common mutation is the mutation of JAK2V617F exon 14. Polycythemia vera can transform into myeloproliferative neoplasm in accelerated phase (MPN-AP) and post polycythemia vera myelofibrosis (PPV-MF). We now report a case of PV transformation into MPN-AP and PPV-MF. A 67-year-old male came to the hospital with fatigue and petechiae on both arms. He was diagnosed with PV in 2013 and has been treated with hydroxyurea 500 mg TID and aspirin 80 mg OD with a satisfactory result. He was diagnosed with myeloproliferative neoplasm in accelerated phase (MPN-AP) in 2018 and had been treated with azacitidine for ten months with a good result. His follow-up bone marrow examination in December 2018 reveals dry tap with fibrosis. He was diagnosed with PPV-MF based on diagnostic criteria from IWG-MRT and given supportive treatment. Polycythemia vera is one of the MPN, with the most common mutation being JAK2V617F. Hydroxyurea is a cytoreductive agent of choice in PV, and its use can speed up the accumulation of mutation inside DNA and, theoretically, increase the risk of transformation. This leukemogenic risk, however still unclear. The transformation from PV to MPN-AP is defined as the 10-19% myeloid blast found within peripheral blood or bone marrow. Several known risk factors of this transformation are unfavorable or complex karyotypes and PV medication. Polycythemia vera also can transform into PPV-MF. This transformation will significantly decrease the life expectancy of patients with a median survival of 5.7 years, with most of the treatments are palliative.

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