Affiliation:
1. Anatomy Unit, Department of Medicine & Surgery (DiMeC), University of Parma, 43126 Parma, Italy
2. University Hospital of Parma, AOU-PR, 43126 Parma, Italy
Abstract
Dysregulated inflammatory signaling is a key feature of myeloproliferative neoplasms (MPNs), most notably of myelofibrosis (MF). Indeed, MF is considered the prototype of onco-inflammatory hematologic cancers. While increased levels of circulatory and bone marrow cytokines are a well-established feature of all MPNs, a very recent body of literature is intriguingly pinpointing the selective overexpression of cytokine receptors by MF hematopoietic stem and progenitor cells (HSPCs), which, by contrast, are nearly absent or scarcely expressed in essential thrombocythemia (ET) or polycythemia vera (PV) cells. This new evidence suggests that MF CD34+ cells are uniquely capable of sensing inflammation, and that activation of specific cytokine signaling axes may contribute to the peculiar aggressive phenotype and biological behavior of this disorder. In this review, we will cover the main cytokine systems peculiarly activated in MF and how cytokine receptor targeting is shaping a novel therapeutic avenue in this disease.
Funder
Azione B—Bando di Ateneo 2021 per la ricerca, University of Parma
Ministero della Salute, Bando Ricerca Finalizzata, sezione Giovani Ricercatori 2021
Subject
General Biochemistry, Genetics and Molecular Biology,Medicine (miscellaneous)