Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms.

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...

Hyper eosinophilia associated with myeloid and lymphoid neoplasms

Eosinophilia in the absence of allergies, asthma, drug reactions, parasitic infections and connective tissue diseases can be eosinophilic clonal disorders, lymphoma or myeloproliferative disorders. Hypereosinophilia with the persistence of eosinophils ≥1500 /mm³ in blood or more than 20% of eosinophils in the bone marrow may be observed in many reactive or clonal disorders, the result of which ...

Myeloproliferative Neoplasms Associated with Mutation in JAK2V617F and Tyrosine Kinase Inhibitors as Therapeutic Strategy

MPNs including a heterogeneous group of clonal or oligoclonal hamtopathies characterized by proliferation and accumulation of mature myeloid cells. JAK2 tyrosine kinase mutation is the most common molecular lesion identified in 90% of cases. JAK2 is involved in EPO signaling pathway, and mutations in it lead to EPO-independent spontaneous phosphorylation. Most tyrosine kinase inhibitors (TKI) a...

Effects of Mutational Combinations on Philadelphia-Negative Myeloproliferative Neoplasms

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...