[Guidelines for the diagnosis and management of paroxysmal nocturnal hemoglobinuria (2024)].

Abstract

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) .