Abstract
Philadelphia negative Myeloproliferative Neoplasms (MPN) are a heterogeneous group of hematopoietic stem cell diseases. MPNs show different risk grades of thrombotic complications and acute myeloid leukemia evolution. In the last couple of decades, from JAK2 mutation detection in 2005 to the newer molecular trademarks studied through next generation sequencing, we are learning to approach MPNs from a deeper perspective. Here, we intend to elucidate the important factors affecting MPN clonal advantage and the reasons why some patients progress to more aggressive disease. Understanding these mechanisms is the key to developing new treatment approaches and targeted therapies for MPN patients.
Highlights
Philadelphia-negative Myeloproliferative Neoplasms (MPN) are heterogeneous hematopoietic stem cell clonal diseases, clinically characterized by an increase of mature hematopoietic peripheral blood cells
According to the 2016 WHO classification [1], MPNs are grouped in polycythemia vera (PV), essential thrombocytopenia (ET), and myelofibrosis (MF)
Drivers mutations as JAK2-V617F, CALR, and MPL occur in the hematopoietic stem cells, causing an MPN phenotype or, in presence of another somatic mutations, to a malignant clonal evolution and progression (Figure 1)
Summary
Philadelphia-negative Myeloproliferative Neoplasms (MPN) are heterogeneous hematopoietic stem cell clonal diseases, clinically characterized by an increase of mature hematopoietic peripheral blood cells. Compared to other myeloid malignancies, PV, ET, and MF have a more chronic course, with prognosis of decades for PV and ET and years for MF. Due to the more available modern molecular biology techniques, such as NGS and Whole Genome Sequencing (WGS), a large number of mutations had been discovered in myeloid malignancies as well as in MPNs, contributing to elucidating new factors influencing the pathogenesis and evolution of these diseases. We intend to take a deeper look into the genetic landscape of MPNs starting from the hematopoietic stem cell (HSC) compartment to understand how this molecular characterization can ameliorate prognosis and treatment of MPN patients
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