Longitudinal inflammatory gene expression associated with advanced myelofibrosis. Longitudinal inflammatory gene expression associated with advanced myelofibrosis

Myelofibrosis (MF) is a progressive, bone marrow (BM) malignancy associated with monocytosis, and is believed to promote the pathological remodelling of the extracellular matrix. Here, we show that the pathologic grading of MF was associated with inflammatory gene expression in primary patient samples. Overall design: The pathology archives at Brigham & Women’s Hospital (BWH) and Massachusetts General Hospital (MGH) was queried to identify patients with myelofibrosis on bone marrow biopsy obtained between 2005 and 2016. Patients diagnosed with myelodysplastic syndrome/MPN overlap disease and those who had progressed to acute leukemia, received treatment with chemotherapeutic agents for prior cancer diagnoses, or had undergone stem cell transplantation were excluded. Histologic review and fibrosis grading were performed by W.W., R.P.H. and O.P. based on consensus, using the 2016 WHO Revised Classification of Myeloid Neoplasms. The study was conducted in accordance with the principles set forth by the Declaration of Helsinki and the requirement for informed consent was waived by the institutional review board. This dataset is an example of data from one patient who progressed from grade 2 to grade 3 myelofibrosis from 2013 to 2016. A separate shared dataset shows single timepoint data from the cohort of patients.

骨髓纤维化（Myelofibrosis, MF）是一种进展性骨髓（bone marrow, BM）恶性血液病，常伴随单核细胞增多症，被认为可促进细胞外基质（extracellular matrix）的病理性重塑。本研究证实，骨髓纤维化的病理分级与患者原发样本中的炎症基因表达水平显著相关。
实验设计：本研究检索了布莱根妇女医院（Brigham & Women’s Hospital, BWH）与麻省总医院（Massachusetts General Hospital, MGH）的病理档案库，筛选出2005年至2016年间行骨髓活检且确诊为骨髓纤维化的患者。本研究排除以下人群：确诊为骨髓增生异常综合征/骨髓增殖性肿瘤（myeloproliferative neoplasms, MPN）重叠疾病者、进展为急性白血病者、因既往恶性肿瘤接受化疗者，或曾接受造血干细胞移植者。组织学复核与纤维化分级由W.W.、R.P.H.及O.P.基于共识完成，采用2016年世界卫生组织（World Health Organization, WHO）修订的《髓系肿瘤分类标准》。本研究严格遵循《赫尔辛基宣言》（Declaration of Helsinki）确立的伦理原则开展，机构审查委员会（institutional review board, IRB）已豁免本研究的知情同意要求。
本数据集为1例患者的随访数据：该患者于2013年至2016年间由2级骨髓纤维化进展为3级骨髓纤维化。另有一份独立共享数据集收录了该研究队列患者的单时间点数据。