Longhitano Lucia, Li Volti Giovanni, Giallongo Cesarina, Spampinato Mariarita, Barbagallo Ignazio, Di Rosa Michelino, Romano Alessandra, Avola Roberto, Tibullo Daniele, Palumbo Giuseppe Alberto
Section of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy.
Department of Scienze Mediche Chirurgiche e Tecnologie Avanzate "G.F. Ingrassia", University of Catania, 95123 Catania, Italy.
J Clin Med. 2020 Jul 22;9(8):2334. doi: 10.3390/jcm9082334.
Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are rare hematological conditions known as myeloproliferative neoplasms (MPNs). They are characterized for being negative malignancies and affected patients often present with symptoms which can significantly impact their quality of life. MPNs are characterized by a clonal proliferation of an abnormal hematopoietic stem/progenitor cell. In MPNs; cells of all myeloid lineages; including those involved in the immune and inflammatory response; may belong to the malignant clone thus leading to an altered immune response and an overexpression of cytokines and inflammatory receptors; further worsening chronic inflammation. Many of these cytokines; in particular, IL-1β and IL-18; are released in active form by activating the inflammasome complexes which in turn mediate the inflammatory process. Despite this; little is known about the functional effects of stem cell-driven inflammasome signaling in MPN pathogenesis. In this review we focused on the role of inflammatory pathway and inflammasome in MPN diseases. A better understanding of the inflammatory-state-driving MPNs and of the role of the inflammasome may provide new insights on possible therapeutic strategies.
真性红细胞增多症（PV）、原发性血小板增多症（ET）和原发性骨髓纤维化（PMF）是罕见的血液疾病，被称为骨髓增殖性肿瘤（MPN）。它们的特征是为阴性恶性肿瘤，患病患者常出现会显著影响其生活质量的症状。MPN的特征是异常造血干/祖细胞的克隆性增殖。在MPN中，所有髓系谱系的细胞，包括参与免疫和炎症反应的细胞，可能都属于恶性克隆，从而导致免疫反应改变以及细胞因子和炎症受体的过度表达，进一步加重慢性炎症。许多这些细胞因子，特别是白细胞介素-1β（IL-1β）和白细胞介素-18（IL-18），通过激活炎性小体复合物以活性形式释放，而炎性小体复合物反过来介导炎症过程。尽管如此，关于干细胞驱动的炎性小体信号在MPN发病机制中的功能作用知之甚少。在本综述中，我们重点关注炎症途径和炎性小体在MPN疾病中的作用。更好地了解驱动MPN的炎症状态以及炎性小体的作用可能为潜在的治疗策略提供新的见解。
