Advances of studies on pathogenesis and management of paroxysmal nocturnal hemoglobinuria / 中国实验血液学杂志
Journal of Experimental Hematology
; (6): 530-535, 2013.
Article
en Zh
| WPRIM
| ID: wpr-332742
Biblioteca responsable:
WPRO
ABSTRACT
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder of hematopoiesis due to the inactivation of PIG-A gene. However, the presence of mutant PIG-A gene in a group of hematopoietic cells is not enough for the development of PNH, immunologic injury and apoptotic effects are considered to play an important role in clonal expansion. Knowledge of the molecular mechanisms leading to PNH has substantially increased in the past decades, which remarkably advances the diagnostic modalities and treatment approaches of patients with PNH. Though great progress has been made because of targeted therapy method, the challenges are still ahead. In this review the advances of studies on mechanism, laboratorial diagnosis and therapeutic protocols of PNH are summarized.
Texto completo:
1
Índice:
WPRIM
Asunto principal:
Patología
/
Terapéutica
/
Proteínas del Sistema Complemento
/
Linfocitos T Reguladores
/
Diagnóstico
/
Genética
/
Hemoglobinuria Paroxística
/
Proteínas de la Membrana
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
/
Guideline
Límite:
Humans
Idioma:
Zh
Revista:
Journal of Experimental Hematology
Año:
2013
Tipo del documento:
Article