Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.
Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, United States.
Front Immunol. 2020 Aug 31;11:2093. doi: 10.3389/fimmu.2020.02093. eCollection 2020.
Philadelphia-negative myeloproliferative neoplasms (MPN) are malignant bone marrow (BM) disorders, typically arising from a single somatically mutated hematopoietic stem cell. The most commonly mutated genes, , , and lead to constitutively active JAK-STAT signaling. Common clinical features include myeloproliferation, splenomegaly and constitutional symptoms. This review covers the contributions of cellular components of MPN pathology (e.g., monocytes, megakaryocytes, and mesenchymal stromal cells) as well as cytokines and soluble mediators to the development of myelofibrosis (MF) and highlights recent therapeutic advances. These findings outline the importance of malignant and non-malignant BM constituents to the pathogenesis and treatment of MF.
费城阴性骨髓增殖性肿瘤（MPN）是一种恶性骨髓（BM）疾病，通常由单个体细胞突变的造血干细胞引起。最常见的突变基因包括 、 、 和 ，导致 JAK-STAT 信号的持续激活。常见的临床特征包括骨髓增殖、脾肿大和全身症状。本综述涵盖了 MPN 病理学的细胞成分（例如单核细胞、巨核细胞和间充质基质细胞）以及细胞因子和可溶性介质对纤维化（MF）发展的贡献，并强调了最近的治疗进展。这些发现概述了恶性和非恶性 BM 成分对 MF 发病机制和治疗的重要性。
