Zhonghua Xue Ye Xue Za Zhi. 2024 Aug 14;45(8):727-737. doi: 10.3760/cma.j.cn121090-20240624-00232.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disorder of hematopoietic stem cells characterized by intravascular hemolysis, bone marrow failure, and a high risk of thrombosis. Although PNH is a benign intravascular hemolytic disease, severe cases may be life-threatening. In recent years, remarkable progress has been made in the diagnosis and treatment of PNH, particularly in the field of complement inhibitor therapy. To further standardize and improve the diagnosis and treatment level of PNH in China, the Red Blood Cell Disease (Anemia) Group of the Chinese Society of Hematology updated the 2013 PNH Expert Consensus by combining the latest diagnosis and treatment progress of PNH, consulting relevant foreign guidelines/consensus and China's national conditions, and soliciting expert advice and opinions to formulate the Chinese Guidelines for the Diagnosis and Treatment of Paroxysmal Nocturnal Hemoglobinuria (2024) .
阵发性睡眠性血红蛋白尿（PNH）是一种罕见的获得性造血干细胞克隆性疾病，其特征为血管内溶血、骨髓衰竭和高血栓形成风险。虽然PNH是一种良性血管内溶血性疾病，但严重病例可能危及生命。近年来，PNH的诊断和治疗取得了显著进展，尤其是在补体抑制剂治疗领域。为进一步规范和提高我国PNH的诊断和治疗水平，中华医学会血液学分会红细胞疾病（贫血）学组结合PNH最新诊断和治疗进展，参考国外相关指南/共识并结合我国国情，征求专家意见，对2013年PNH专家共识进行更新，制定了《阵发性睡眠性血红蛋白尿诊断与治疗中国指南（2024年版）》。
