The Genetic And Clinical Risk Factors For Explosion Phase Progression Of Chronic Leukemia

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Introduction

The myeloproliferative neoplasms carry an inherent risk of transformation to an accelerated-phase disease, as well as ultimately to acute leukemia. The likelihood of disease evolution is mitigated by various clinical, biological and genetic factors, as well as the by the underlying initial MPN diagnosis. Here we review the genetic and clinical risk factors for blast-phase progression of chronic-phase Phliadelphia-chromosome negative MPNs, as well as therapeutic outcomes to date. We also review the key pathologic findings in disease evolution and transformation

Definitions of disease progression and presentation The definition of acute leukemia arising from an antecedent MPN follows standard WHO convention for the diagnosis of acute leukemia. The presence of greater than or equal to 20% blasts in the peripheral blood or bone marrow fulfills this definition. Notably, a discrepancy between bone marrow and peripheral blood blasts is frequently observed in patients with preceding MPN. In many cases this is due to fibrosis of the marrow which renders a bone marrow aspirate unobtainable. As well, it has been postulated that areas of extramedullary hematopoiesis contribute to leukemogensis which may lead to further differences between blast counts observed in the bone marrow and peripheral blood (1). Notably, the International Working Group for Myelofibrosis Research and Treatment (IWG-MRT) has proposed nomenclature for patients with post-ET MF, post-PV MF and PMF

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