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Molecular Vision of Life

Although the clinical presentations of the 3 MPN entities are different in their typical forms, establishment of precise diagnosis at disease onset is often challenging. This is reflected by the 2016 revision of the World Health Organization (WHO) diagnostic criteria for MPN.2 In many cases, a continuum between these disease subtypes can be observed, as documented by the progression of ET and PV to secondary myelofibrosis (MF). Furthermore, boundaries between these 3 disorders cannot be well established, especially between ET and PMF. Thus, transitional entities may emerge describing disease states such as prefibrotic PMF (or early PMF) that displays an ET phenotype at diagnosis with typical bone marrow histology, but with a high probability of progression to MF and worse prognosis than true ET.3