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
in Chinese
| 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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pathology
/
Therapeutics
/
Complement System Proteins
/
T-Lymphocytes, Regulatory
/
Diagnosis
/
Genetics
/
Hemoglobinuria, Paroxysmal
/
Membrane Proteins
Type of study:
Diagnostic study
/
Etiology study
/
Practice guideline
Limits:
Humans
Language:
Chinese
Journal:
Journal of Experimental Hematology
Year:
2013
Type:
Article
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