[Expert consensus on clonal screening and monitoring of complement inhibitor therapy in paroxysmal nocturnal hemoglobinuria (2024)].
Zhonghua Xue Ye Xue Za Zhi
; 45(2): 109-114, 2024 Feb 14.
Article
em Zh
| MEDLINE
| ID: mdl-38604785
ABSTRACT
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease with abnormal hematopoietic stem cells that causes intravascular hemolytic anemia, thrombosis, and peripheral blood cytopenia. It has a chronic progressive course and can be fatal in severe cases if not treated aggressively. Complement inhibitors are the first-line recommended treatment for hemolysis-related symptoms of PNH. With the rapid development of new complement inhibitors, it is critical to quickly screen and confirm the diagnosis, identify patients with complement inhibitor indications, and monitor breakthrough hemolysis and extravascular hemolysis during complement inhibitor therapy. Drawing on the most recent guidelines, works of literature, and meta-reviews from around the world, as well as combining with experience from the experts, this consensus focused on PNH screening principles, the significance of PNH cloning detection, and post-treatment monitoring of terminal complement inhibitors, which may contribute to a better understanding of diagnosis and treatment monitoring in the era of complement inhibitors.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Hemoglobinúria Paroxística
Limite:
Humans
Idioma:
Zh
Revista:
Zhonghua Xue Ye Xue Za Zhi
Ano de publicação:
2024
Tipo de documento:
Article