The classic BCR-ABL1-negative myeloproliferative neoplasm is an operational sub-category of MPNs that includes polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The JAK2V617F mutation is found in ~ 95% of PV and 50-60% of ET or PMF. In most of the remaining JAK2V617F- negative PV cases, JAK2 exon 12 mutations are present. Amongst the JAK2V617F-negative ET ...

The activating JAK2V617F mutation has been described in the majority of patients with BCR-ABL-negative myeloproliferative disorders (MPD). In this report, we characterize the small-molecule LS104 as a novel non-ATP-competitive JAK2 inhibitor: Treatment of JAK2V617F-positive cells with LS104 resulted in dose-dependent induction of apoptosis and inhibition of JAK2 autophosphorylation and of downs...

In this issue of Blood, Chen et al and Kameda et al demonstrate that Tet2 loss has 2 effects in Jak2V617F mice: it increases both the severity of the myeloproliferative disorders and the self-renewal properties of the Jak2V617F hematopoietic stem cells (HSCs).

To the Editor: Essential thrombocythemia (ET) complicated by cerebral venous sinus thrombosis (CVST) is exceptionally rare. Hitherto, few studies have mentioned the role of Janus kinase 2V617F (JAK2V617F) mutation in ET patients who developed CVST; thus, the strategy and long‐term outcomes are yet rarely reported. The present study documented four cases, wherein CVST was the first manifestation...

JAK2V617F mutation is found in about 60% of cases of essential thrombocytemia (ET) and represents a driving mutation. Immune thrombocytopenia (ITP) is an autoimmune disease characterized by a low platelet (PLT) count. So far, only 2 reports described ET following ITP. For the first time we analyzed in a patient the JAK2V617F allele burden at ITP onset occurred 13 years before the ET diagnosis a...

BACKGROUND The identification of the JAK2V617F mutation is mandatory in the diagnostic work-up of Philadelphia chromosome-negative myeloproliferative neoplasms. Several molecular techniques to detect this mutation are currently available, but each of them has some limits. DESIGN AND METHODS We set up a novel molecular method for the identification of the JAK2V617F mutation based on an allele-...

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 (...

PURPOSE The influence of JAK2 V617F mutation on blast transformation (BT) and overall survival (OS) in primary myelofibrosis (PMF) is controversial. In a large cohort of patients we applied competing risks analysis for studying the influence of JAK2V617F mutation on BT in PMF. PATIENTS AND METHODS In 462 PMF-fibrotic type patients (bone marrow [BM] fibrosis grade >0) we computed the incidence...

BACKGROUND The driver mutations JAK2V617F, MPLW515L/K and CALR influence disease phenotype of myeloproliferative neoplasms (MPNs) and might sustain a condition of chronic inflammation. Pentraxin 3 (PTX3) and high-sensitivity C-reactive protein (hs-CRP) are inflammatory biomarkers potentially useful for refining prognostic classification of MPNs. METHODS We evaluated 305 with essential thrombo...