Research Advances of Acquired Thrombotic Thrombocytopenic Purpura--Review.
10.7534/j.issn.1009-2137.2018.04.048
- Author:
Xi JIA
1
;
Yang HE
1
;
Chang-Geng RUAN
2
Author Information
1. Jiangsu Institute of Hematology , The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China.
2. Jiangsu Institute of Hematology , The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China E-mail: heyang1963@163.com.
- Publication Type:Journal Article
- MeSH:
ADAMTS13 Protein;
Anemia, Hemolytic;
Blood Platelets;
Humans;
Purpura, Thrombotic Thrombocytopenic;
von Willebrand Factor
- From:
Journal of Experimental Hematology
2018;26(4):1230-1234
- CountryChina
- Language:Chinese
-
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.
