Hematology Unit, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", 47014 Meldola, Italy.
Biosciences Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", 47014 Meldola, Italy.
Int J Mol Sci. 2021 Jan 8;22(2):561. doi: 10.3390/ijms22020561.
The last decade has been very important for the quantity of preclinical information obtained regarding chronic myeloproliferative neoplasms (MPNs) and the following will be dedicated to the translational implications of the new biological acquisitions. The overcoming of the mechanistic model of clonal evolution and the entry of chronic inflammation and dysimmunity into the new model are the elements on which to base a part of future therapeutic strategies. The innate immune system plays a major role in this context. Protagonists of the initiation and regulation of many pathological aspects, from cytokine storms to fibrosis, the NLRP3 and AIM2 inflammasomes guide and condition the natural history of the disease. For this reason, MPNs share many biological and clinical aspects with non-neoplastic diseases, such as autoimmune disorders. Finally, cardiovascular risk and disturbances in iron metabolism and myelopoiesis are also closely linked to the role of inflammasomes. Although targeted therapies are already being tested, an increase in knowledge on the subject is desirable and potentially translates into better care for patients with MPNs.
过去十年在获得有关慢性骨髓增生性肿瘤（MPN）的临床前信息方面非常重要，以下将专门讨论新生物学发现的转化意义。克服克隆进化的机制模型并将慢性炎症和免疫失调纳入新模型是未来治疗策略的基础的一部分。先天免疫系统在这方面起着重要作用。在许多病理方面（从细胞因子风暴到纤维化）的起始和调节中发挥重要作用的 NOD 样受体蛋白 3（NLRP3）和 AIM2 炎性小体，指导并影响疾病的自然病程。出于这个原因，MPN 与非肿瘤性疾病（如自身免疫性疾病）具有许多生物学和临床方面的共同特征。最后，心血管风险以及铁代谢和髓系细胞生成紊乱也与炎性小体的作用密切相关。尽管已经在测试靶向治疗，但增加对该主题的了解是可取的，并可能转化为为 MPN 患者提供更好的护理。
