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,其特征为慢性炎症状态(表现为具有预后意义的高水平炎症细胞因子及持续的氧化应激,这已被证实与疾病发病机制密切相关)以及骨髓和脾脏中广泛的新生血管形成。髓源性抑制细胞(MDSCs)是在癌症、脓毒症或慢性炎症患者体内扩增的未成熟细胞,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。本文评估了PMF患者循环MDSCs的存在情况、参与其动员/扩增的血浆因子,以及与实验室、遗传和临床参数的相关性。数据表明,MDSCs可能在PMF中具有重要作用,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,或可作为个体化治疗的潜在新靶点。