The immune landscape in BCR-ABL negative myeloproliferative neoplasms: inflammation, infections and opportunities for immunotherapy.
The immune landscape in BCR-ABL negative myeloproliferative neoplasms: inflammation, infections and opportunities for immunotherapy.
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DOI：
10.1111/bjh.17850
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发表时间：
2022-03
影响因子：
6.5
通讯作者：
Psaila, Bethan
中科院分区：
文献类型：
--
作者：
Strickland, Marie;Quek, Lynn;Psaila, Bethan
断点簇区- Abelson （BCR - ABL）阴性骨髓增生性肿瘤（mpn）是由造血干细胞获得基因突变引发的慢性髓系肿瘤，导致克隆扩增和血细胞及其祖细胞的过量产生。mpn包括一系列疾病，具有重叠但不同的分子、实验室和临床特征。这包括真性红细胞增多症、原发性血小板增多症和骨髓纤维化。免疫系统失调是mpn病理的关键，支持克隆进化，介导症状并导致不同程度的免疫功能低下。靶向免疫功能障碍是一种重要的治疗策略。在本综述中，我们将重点关注mpn患者的免疫景观-炎症在疾病发病机制中的作用，对感染的易感性以及治疗性免疫调节的新策略。需要进一步详细的工作来更精确地描述MPN中的免疫扰动，以确定临床亚型之间对感染的易感性如何以及为什么不同，并更好地理解炎症如何导致MPN克隆的竞争优势。这些研究可能有助于揭示疾病修饰疗法的新设计。
Breakpoint cluster region‐Abelson (BCR‐ABL) negative myeloproliferative neoplasms (MPNs) are chronic myeloid neoplasms initiated by the acquisition of gene mutation(s) in a haematopoietic stem cell, leading to clonal expansion and over‐production of blood cells and their progenitors. MPNs encompass a spectrum of disorders with overlapping but distinct molecular, laboratory and clinical features. This includes polycythaemia vera, essential thrombocythaemia and myelofibrosis. Dysregulation of the immune system is key to the pathology of MPNs, supporting clonal evolution, mediating symptoms and resulting in varying degrees of immunocompromise. Targeting immune dysfunction is an important treatment strategy. In the present review, we focus on the immune landscape in patients with MPNs – the role of inflammation in disease pathogenesis, susceptibility to infection and emerging strategies for therapeutic immune modulation. Further detailed work is required to delineate immune perturbation more precisely in MPNs to determine how and why vulnerability to infection differs between clinical subtypes and to better understand how inflammation results in a competitive advantage for the MPN clone. These studies may help shed light on new designs for disease‐modifying therapies.
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影响因子：
11.2
作者：
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通讯作者：
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