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MYELOFIBROSIS IN A TREATED CASE OF POLYCYTHEMIA VERA

Author: Arunima Deb, MD, 12/01/2020
Category: Myeloid Neoplasms and acute leukemia (WHO 2016) > Myeloproliferative Neoplasms (MPN) > Polycythemia Vera (PV) > Post-polycythemic myelofibrosis
Published Date: 12/02/2020

The patient is a 71-year-old female with a history of polycythemia vera (PV) with JAK2 mutation diagnosed in 2014 without further follow-up regarding treatment. She presented to our hospital in October 2020 with shortness of breath. She was found to have myeloid blast transformation with 22% blasts on peripheral blood with underlying TET2, TP53 and ASXL1 mutation. The patient was enrolled in a clinical trial with decitabine and venetoclax and is undergoing bone marrow biopsy after a cycle of therapy.

The images depicted below are typical of an end-stage fibrotic phase Ph-negative myeloproliferative neoplasms. While early non-fibrotic stages maybe distinguished easily on morphologic grounds, end-stage fibrotic phase of MPNs uniformly demonstrate mild osteosclerosis, fibrosis and hyperchromatic megakaryocytes as noted here.