Chinese guidelines on the diagnosis and treatment of adult essential thrombocythemia (2026)

Essential thrombocythemia (ET) is a classic Philadelphia chromosome-negative myeloproliferative neoplasm characterized by excessive proliferation of bone marrow megakaryocytes and sustained elevation of peripheral blood platelet counts. The primary clinical risks include thrombotic and bleeding events, with a minority of patients experiencing the risk of progression to myelofibrosis or transformation to acute leukemia. In recent years, the demand for standardized diagnosis and treatment of ET has become increasingly prominent with advances in molecular testing, bone marrow pathology diagnosis, novel drug development, and clinical management. To provide actionable clinical practice guidance for hematologists in China, the Thrombosis and Hemostasis Group of the Chinese Society of Hematology, Chinese Medical Association, convened domestic experts to formulate this guideline, based on both international and domestic evidence-based medical evidence combined with expert consensus.

原发性血小板增多症（essential thrombocythemia，ET）是一类以骨髓巨核细胞过度增殖及外周血血小板计数持续增高为特征的经典型费城染色体阴性骨髓增殖性肿瘤，主要临床风险为血栓与出血事件，少数患者存在进展为骨髓纤维化或急性白血病的风险。近年来，随着分子检测、骨髓病理诊断、新药研发及临床管理水平的提升，ET规范化诊疗的需求日益突出。为向我国血液科医师提供可操作的临床实践指导，中华医学会血液学分会血栓与止血学组组织国内相关专家，基于国内外循证医学证据并结合专家共识，制订了本指南。.