Increased Pentraxin-3 Correlates With The Activity Of The Neoplastic Clone In Primary Myelofibrosis

Background: Primary myelofibrosis (PMF) refers to a fatal myeloproliferative neoplasm characterized by progressive bone marrow (BM) fibrosis. A well-described feature of PMF is an increase in circulating cytokines, which induce a systemic inflammatory state with debilitating constitutional symptoms. Treatment with JAK inhibitors has significantly improved the quality of life of PMF patients, acting predominantly through the inhibition of cytokine production. However, the effects of such agents on the reversal of the BM fibrosis have remained limited. Recently, members of the pentraxin family of circulating immune proteins have been associated with the regulation of tissue remodeling in the BM of PMF patients. Specifically, pentraxin-3 (PTX3) has been shown to promote the differentiation of monocytes to collagen-producing fibrocytes in vitro and in vivo . In contrast to the liver-secreted short pentraxins, PTX3 is produced by myeloid, mesenchymal and endothelial cells, suggesting a potentially paramount role in inducing the BM fibrosis. Here, we tested the hypothesis that the plasma levels of PTX3 are increased in the PMF patients and that they can be correlated with the activity of the neoplastic clone over the course of the disease.