Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.

Details

Serval ID
serval:BIB_8F7A6F0D072E
Type
Article: article from journal or magazin.
Collection
Publications
Title
Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
Journal
Blood
Author(s)
Bianchi V., Robles R., Alberio L., Furlan M., Lämmle B.
ISSN
0006-4971 (Print)
ISSN-L
0006-4971
Publication state
Published
Issued date
2002
Peer-reviewed
Oui
Volume
100
Number
2
Pages
710-713
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Abstract
A severe deficiency in von Willebrand factor-cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a diagnosis of hemolytic uremic syndrome. However, ADAMTS13 deficiency has been claimed not to be specific for TTP, since it was observed in various thrombocytopenic and other conditions. We studied 68 patients with thrombocytopenia due to severe sepsis or septic shock (n = 17), heparin-induced thrombocytopenia (n = 16), idiopathic thrombocytopenic purpura (n = 10), or other hematologic (n = 15) or miscellaneous conditions (n = 10). Twelve of the 68 patients had subnormal levels of ADAMTS13 activity (<or= 30%), but none had less than 10%. Thus, the study showed that ADAMTS13 activity is decreased in a substantial proportion of patients with thrombocytopenia of various causes. A severe deficiency of ADAMTS13 (< 5%), identified in more than 120 patients during 1996 to 2001 in our laboratory, is specific for a thrombotic microangiopathy commonly labeled TTP.
Keywords
ADAM Proteins, Adult, Aged, Aged, 80 and over, Biological Markers/blood, Female, Humans, Male, Metalloendopeptidases/blood, Metalloendopeptidases/deficiency, Middle Aged, Purpura, Thrombotic Thrombocytopenic/enzymology, Retrospective Studies, Thrombocytopenia/enzymology
Pubmed
Web of science
Open Access
Yes
Create date
10/02/2015 11:04
Last modification date
20/08/2019 14:53
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