Campanelli Rita, Carolei Adriana, Catarsi Paolo, Abbà Carlotta, Boveri Emanuela, Paulli Marco, Gentile Raffaele, Morosini Monica, Albertini Riccardo, Mantovani Stefania, Massa Margherita, Barosi Giovanni, Rosti Vittorio
Center for the Study of Myelofibrosis, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.
General Medicine 2-Center for Systemic Amyloidosis and High-Complexity Diseases, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.
Cancers (Basel). 2024 Jul 16;16(14):2556. doi: 10.3390/cancers16142556.
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by a chronic inflammatory state that plays a relevant role in the disease pathogenesis (as proven by high levels of inflammatory cytokines with prognostic significance and by a persistent oxidative stress) and by extensive neoangiogenesis in bone marrow (BM) and spleen. Myeloid-derived suppressor cells (MDSCs) are immature cells that expand in patients with cancer, sepsis or chronic inflammation, favoring tumor onset and progression mainly through the decrease in immune surveillance and the promotion of neoangiogenesis. In this paper, we evaluated the presence of circulating MDSCs in PMF patients, the plasmatic factors involved in their mobilization/expansion and the correlations with laboratory, genetic and clinical parameters. The data indicated that MDSCs could have a relevant role in PMF as a new pathogenic mechanism contributing to explaining the phenotypic diversity observed during the clinical course of the disease, or a potential new target for personalized treatment.
原发性骨髓纤维化（PMF）是一种骨髓增殖性肿瘤，其特征在于慢性炎症状态，该状态在疾病发病机制中起重要作用（高水平具有预后意义的炎性细胞因子以及持续的氧化应激证明了这一点），并且在骨髓（BM）和脾脏中存在广泛的新生血管形成。髓系来源的抑制细胞（MDSC）是在癌症、败血症或慢性炎症患者中扩增的未成熟细胞，主要通过降低免疫监视和促进新生血管形成来促进肿瘤的发生和进展。在本文中，我们评估了PMF患者循环中MDSC的存在情况、参与其动员/扩增的血浆因子以及与实验室、基因和临床参数的相关性。数据表明，MDSC可能在PMF中起重要作用，作为一种新的致病机制，有助于解释疾病临床过程中观察到的表型多样性，或者作为个性化治疗的潜在新靶点。
