Document Detail


Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
MedLine Citation:
PMID:  12091372     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Valentina Bianchi; Rodolfo Robles; Lorenzo Alberio; Miha Furlan; Bernhard Lämmle
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Blood     Volume:  100     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  2002 Jul 
Date Detail:
Created Date:  2002-07-01     Completed Date:  2002-08-30     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  710-3     Citation Subset:  AIM; IM    
Affiliation:
Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.
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MeSH Terms
Descriptor/Qualifier:
ADAM Proteins
Adult
Aged
Aged, 80 and over
Biological Markers / blood
Female
Humans
Male
Metalloendopeptidases / blood,  deficiency*
Middle Aged
Purpura, Thrombotic Thrombocytopenic / enzymology
Retrospective Studies
Thrombocytopenia / enzymology*
Chemical
Reg. No./Substance:
0/Biological Markers; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human; EC 3.4.24.-/Metalloendopeptidases
Comments/Corrections
Comment In:
Blood. 2002 Nov 15;100(10):3839-40; author reply 3840-2   [PMID:  12411328 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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