Research Advances of Acquired Thrombotic Thrombocytopenic Purpura--Review / 中国实验血液学杂志
Journal of Experimental Hematology
; (6): 1230-1234, 2018.
Article
de Zh
| WPRIM
| ID: wpr-689500
Bibliothèque responsable:
WPRO
ABSTRACT
Thrombotic thrombocytopenic purpura (TTP) is an acute, potentially life-threatening thrombotic microangiopathies (TMA). TTP has mainly been diagnosed by clinical findings, such as thrombocytopenia and microangiopathic haemolytic anemia. In addition, the reduced activity of von Willebrand factor-cleaving metalloproteinase ADAMTS13 below 10% has been accepted internationally as a diagnostic criterion for TTP. In clinic, the accurate diagnosis and early initiation of therapy can significantly improve the survival rate of patients. Therapy should be focused on increasing ADAMTS13 activity and eliminating or inhibiting ADAMTS13 antibody by the infusion or exchange of therapeutic plasma and corticosteroids. Both the administration of recombinant ADAMTS13 and reducing the release of human neutrophil peptides (HNPs) would be novel promising strategies for the prevention of platelet-vWFinteraction. This review briefly summarizes the recent advances in terms of pathogenesis, diagnosis and treatment of TTP.
Texte intégral:
1
Indice:
WPRIM
Sujet Principal:
Purpura thrombotique thrombocytopénique
/
Plaquettes
/
Facteur de von Willebrand
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Protéine ADAMTS13
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Anémie hémolytique
Limites du sujet:
Humans
langue:
Zh
Texte intégral:
Journal of Experimental Hematology
Année:
2018
Type:
Article