Genetics and Pathogenetic Role of Inflammasomes in Philadelphia Negative Chronic Myeloproliferative Neoplasms: A Narrative Review.

Genetics and Pathogenetic Role of Inflammasomes in Philadelphia Negative Chronic Myeloproliferative Neoplasms: A Narrative Review.
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DOI:
10.3390/ijms22020561
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发表时间:
2021-01-08
影响因子:
5.6
通讯作者:
Lucchesi A
Lucchesi A
中科院分区:
生物学2区
文献类型:
--
作者:
Di Battista V;Bochicchio MT;Giordano G;Napolitano M;Lucchesi A

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过去十年对于获得有关慢性骨髓增殖性肿瘤(MPN)的临床前信息的数量非常重要,接下来的十年将致力于新的生物收购的转化影响。克服克隆进化的机制模型以及将慢性炎症和免疫失调纳入新模型是未来治疗策略的一部分的基础要素。先天免疫系统在这方面发挥着重要作用。 NLRP3 和 AIM2 炎症小体是从细胞因子风暴到纤维化等许多病理方面的启动和调节的主角,指导和调节疾病的自然史。因此,MPN 与非肿瘤性疾病(例如自身免疫性疾病)有许多生物学和临床方面的共同点。最后,心血管风险以及铁代谢和骨髓生成的紊乱也与炎症小体的作用密切相关。尽管靶向治疗已经在进行测试,但增加有关该主题的知识是可取的,并且有可能转化为对 MPN 患者更好的护理。
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.
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