effects of mutational combinations on philadelphia-negative myeloproliferative neoplasms

Effects of Mutational Combinations on Philadelphia-Negative Myeloproliferative Neoplasms

Effects of Mutational Combinations on Philadelphia-Negative Myeloproliferative Neoplasms

Iran J Med Sci March 2017; Vol 42 No 2 219 Dear Editor, Philadelphia-negative myeloproliferative neoplasms (MPNs) were first described 65 years ago. Yet, the molecular features of the disease have become of interest since 2005 following the identification of the JAK2V617F mutation.1 Between 90% and 98% of patients with polycythemia vera and about 50% of patients with essential thrombocythemia (...

Philadelphia-negative chronic myeloproliferative neoplasms

Chronic myeloproliferative diseases without the Philadelphia chromosome marker (Ph-), although first described 60 years ago, only became the subject of interest after the turn of the millennium. In 2001, the World Health Organization (WHO) defined the classification of this group of diseases and in 2008 they were renamed myeloproliferative neoplasms based on morphological, cytogenetic and molec...

Molecular biology of Philadelphia-negative myeloproliferative neoplasms

Myeloproliferative neoplasms are clonal diseases of hematopoietic stem cells characterized by myeloid hyperplasia and increased risk of developing acute myeloid leukemia. Myeloproliferative neoplasms are caused, as any other malignancy, by genetic defects that culminate in the neoplastic phenotype. In the past six years, since the identification of JAK2V617F, we have experienced a substantial i...

New knowledge of Philadelphia Negative Classical Myeloproliferative Neoplasms

This article tries to answer the following questions: why we are in need of a revision of the prior diagnostic criteria for Philadelphia -ve MPNs, what is new in 2016 multiparameter WHO classification for Philadelphia-ve MPNs and how a single JAK2V617F mutation can lead to three different MPNs. In addition it discusses the genetic basis and molecular pathophysiology of Philadelphia-ve classical...

Evolving Therapeutic Strategies for the Classic Philadelphia-Negative Myeloproliferative Neoplasms

Despite the emergence of JAK inhibitors, there is a need for disease-modifying treatments for Philadelphia-negative myeloproliferative neoplasms (MPNs). JAK inhibitors ameliorate symptoms and address splenomegaly, but because of the heterogeneous contributors to the disease process, JAK inhibitor monotherapy incompletely addresses the burden of disease. The ever-growing understanding of MPN pat...