费城染色体阴性的慢性髓增生性肿瘤（MPNs）是由于干细胞中获得性体细胞驱动突变而产生，从最初的癌症阶段（特发性血小板增多症、红细胞增多症）经过10-30年的发展进展到骨髓纤维化晚期并出现骨髓衰竭。JAK2V617F突变是最常见的驱动突变。慢性炎症被认为是主要的发病因素，既是MPN发展的触发因素，也是疾病进展的驱动因素。MPN的慢性炎症表现为持久的结缔组织重塑，导致器官功能受损且最终器官衰竭，因为细胞外基质（ECM）过度积累。考虑到MPN是在炎症微环境中发展的获得性克隆干细胞疾病，在这种环境中造血细胞群体被间质增生逐渐代替，我们的目标是在此提供关于循环ECM片段在MPN的诊断、治疗和监测中的综述。我们探讨了使用循环ECM蛋白片段作为生物学上可行的非侵入性疾病标志物在MPN管理中的合理性，并突出了新的视角。

Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPNs) arise due to acquired somatic driver mutations in stem cells and develop over 10-30 years from the earliest cancer stages (essential thrombocythemia, polycythemia vera) towards the advanced myelofibrosis stage with bone marrow failure. The JAK2V617F mutation is the most prevalent driver mutation. Chronic inflammation is considered to be a major pathogenetic player, both as a trigger of MPN development and as a driver of disease progression. Chronic inflammation in MPNs is characterized by persistent connective tissue remodeling, which leads to organ dysfunction and ultimately, organ failure, due to excessive accumulation of extracellular matrix (ECM). Considering that MPNs are acquired clonal stem cell diseases developing in an inflammatory microenvironment in which the hematopoietic cell populations are progressively replaced by stromal proliferation-"a wound that never heals"-we herein aim to provide a comprehensive review of previous promising research in the field of circulating ECM fragments in the diagnosis, treatment and monitoring of MPNs. We address the rationales and highlight new perspectives for the use of circulating ECM protein fragments as biologically plausible, noninvasive disease markers in the management of MPNs.