Murphy I G, Mitchell E L, Raso-Barnett L, Godfrey A L, Godfrey E M
Department of Radiology, Addenbrooke's Hospital, Hills Rd, Cambridge CB2 0QQ, UK.
Department of Haematology, Addenbrooke's Hospital, Hills Rd, Cambridge CB2 0QQ, UK.
Clin Radiol. 2017 Oct;72(10):801-809. doi: 10.1016/j.crad.2017.05.014. Epub 2017 Jun 12.
Myeloproliferative neoplasms (MPNs) are a heterogeneous group of haematological disorders including polycythaemia vera (PV), essential thrombocythaemia (ET), primary myelofibrosis (PMF), and chronic myeloid leukaemia (CML). These disorders show large overlap in genetic and clinical presentations, and can have many different imaging manifestations. Unusual thromboses, embolic events throughout the systemic or pulmonary vasculature, or osseous findings can often be clues to the underlying disease. There is limited literature about the imaging features of these disorders, and this may result in under-diagnosis. Multiple treatments are available for symptom control, and the development of multiple new pharmacological inhibitors has significantly improved morbidity and prognosis. Knowledge of these conditions may enable the radiologist to suggest an MPN as a possible underlying cause for certain imaging findings, particularly unexplained splanchnic venous thrombosis, i.e. in the absence of chronic liver disease or pancreatitis. The aim of the present review is to outline using examples the different categories of MPN and illustrate the variety of radiological findings associated with these diseases.
骨髓增殖性肿瘤(MPN)是一组异质性血液系统疾病,包括真性红细胞增多症(PV)、原发性血小板增多症(ET)、原发性骨髓纤维化(PMF)和慢性髓性白血病(CML)。这些疾病在基因和临床表现上有很大重叠,并且可能有许多不同的影像学表现。不寻常的血栓形成、全身或肺血管系统的栓塞事件或骨质表现常常是潜在疾病的线索。关于这些疾病影像学特征的文献有限,这可能导致诊断不足。有多种治疗方法可用于控制症状,多种新型药理抑制剂的开发显著改善了发病率和预后。了解这些疾病可能使放射科医生能够将MPN作为某些影像学表现的潜在病因提出,特别是不明原因的内脏静脉血栓形成,即在没有慢性肝病或胰腺炎的情况下。本综述的目的是通过实例概述MPN的不同类别,并说明与这些疾病相关的各种放射学表现。