Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute.
Stanford Cancer Institute.
J Natl Compr Canc Netw. 2022 Sep;20(9):1033-1062. doi: 10.6004/jnccn.2022.0046.
The classic Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) consist of myelofibrosis, polycythemia vera, and essential thrombocythemia and are a heterogeneous group of clonal blood disorders characterized by an overproduction of blood cells. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for MPN were developed as a result of meetings convened by a multidisciplinary panel with expertise in MPN, with the goal of providing recommendations for the management of MPN in adults. The Guidelines include recommendations for the diagnostic workup, risk stratification, treatment, and supportive care strategies for the management of myelofibrosis, polycythemia vera, and essential thrombocythemia. Assessment of symptoms at baseline and monitoring of symptom status during the course of treatment is recommended for all patients. This article focuses on the recommendations as outlined in the NCCN Guidelines for the diagnosis of MPN and the risk stratification, management, and supportive care relevant to MF.
经典的费城染色体阴性骨髓增殖性肿瘤（MPN）包括骨髓纤维化、真性红细胞增多症和原发性血小板增多症，是一组异质性克隆性血液疾病，其特征为血细胞过度生成。NCCN 肿瘤学临床实践指南（NCCN 指南）是由多学科专家小组召集会议制定的，目的是为成人 MPN 的管理提供建议。该指南包括骨髓纤维化、真性红细胞增多症和原发性血小板增多症的诊断性评估、风险分层、治疗和支持治疗策略的建议。建议对所有患者进行基线症状评估，并在治疗过程中监测症状状态。本文重点介绍 NCCN 指南中关于 MPN 的诊断以及与 MF 相关的风险分层、管理和支持治疗的建议。
