Interpretation of the guidelines for diagnosing and treating paroxysmal nocturnal hemoglobinuria in China (2024)
10.3760/cma.j.issn.0253-2727.2024.12.101
- VernacularTitle:阵发性睡眠性血红蛋白尿症诊断与治疗中国指南（2024年版）解读
- Author:
Liyan LI
1
;
Rong FU
1
Author Information
1. 天津医科大学总医院，天津市骨髓衰竭及癌性造血克隆防治重点实验室，天津市血液病研究所，天津 300052
- Publication Type:Journal Article
- From:
Chinese Journal of Hematology
2024;45(12):1071-1077
- CountryChina
- Language:Chinese
-
Abstract:
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder of hematopoietic stem cells induced by PIG-A gene mutations. It is clinically manifested by hemolysis, bone marrow failure, and high-risk concurrent thrombosis, which are life-threatening in severe cases. Significant progress has been made in the pathogenesis research and clinical diagnosis and treatment of PNH in recent years. The Red Blood Cell Disease (Anemia) Group Chinese Society of Hematology, Chinese Medical Association, combined with the latest diagnosis and treatment progress of PNH, relevant foreign guidelines/consensus, and China’s national conditions, jointly formulated the "Guidelines for the diagnosis and management of paroxysmal nocturnal hemoglobinuria (2024) " based on extensive solicitation of expert suggestions and opinions. This article discussed the key and difficult issues in PNH diagnosis and treatment, interpreted the updated part of the guidelines, and expanded the relevant recommendations of the guidelines according to the latest research progress at home and abroad, thereby providing more references for clinical practice.
