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IRF4 expression is low in Philadelphia negative myeloproliferative neoplasms and is associated with a worse prognosis
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IRF4 expression is low in Philadelphia negative myeloproliferative neoplasms and is associated with a worse prognosis
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Journal Article
IRF4 expression is low in Philadelphia negative myeloproliferative neoplasms and is associated with a worse prognosis
2021
Overview
Interferon regulatory factor 4 (
IRF4
) is involved in the pathogenesis of various hematologic malignancies. Its expression has been related to the negative regulation of myeloid-derived suppressor cells (MDSCs) and the polarization of anti-inflammatory M2 macrophages, thereby altering immunosurveillance and inflammatory mechanisms. An abnormal inflammatory status in the bone marrow microenvironment of myeloproliferative neoplasms (MPNs) has recently been demonstrated; moreover, in chronic myeloid leukemia a downregulated expression of
IRF4
has been found. In this context, we evaluated the
IRF4
expression in 119 newly diagnosed consecutive Philadelphia negative MPNs (Ph- MPNs), showing a low expression among the MPNs phenotypes with a more significant decrease in primary myelofibrosis patients. Lower
IRF4
levels were associated with
JAK2
+ and triple negatives cases carrying the worst prognosis. Furthermore, the
IRF4
levels were related to leukemic transformation and a shorter leukemia-free survival; moreover, the risk of myelofibrosis transformation in polycythemia vera and essential thrombocythemia patients was more frequent in cases with lower
IRF4
levels. Overall, our study demonstrates an
IRF4
dysregulated expression in MPNs patients and its association with a worse prognosis. Further studies could validate these data, to improve our knowledge of the MPNs pathogenesis and confirm the
IRF4
role as a new prognostic factor.
Publisher
BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
