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.
Artigo
em Chinês
| WPRIM
| ID: wpr-332742
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:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Patologia
/
Terapêutica
/
Proteínas do Sistema Complemento
/
Linfócitos T Reguladores
/
Diagnóstico
/
Genética
/
Hemoglobinúria Paroxística
/
Proteínas de Membrana
Tipo de estudo:
Estudo diagnóstico
/
Estudo de etiologia
/
Guia de Prática Clínica
Limite:
Humanos
Idioma:
Chinês
Revista:
Journal of Experimental Hematology
Ano de publicação:
2013
Tipo de documento:
Artigo
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