Document Detail


Deficiency of von Willebrand factor-cleaving protease in familial and acquired thrombotic thrombocytopenic purpura.
MedLine Citation:
PMID:  10097823     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Excessive intravascular platelet agglutination in patients with thrombotic thrombocytopenic purpura (TTP) appears to be associated with excessive release from endothelial cells of unusually large von Willebrand factor (vWF) multimers and/or impaired degradation of these multimers by a 'depolymerase' cleaving vWF to smaller, non-agglutinating molecular forms. We studied the activity of a recently described vWF-cleaving protease in four patients, including two brothers, with chronic relapsing TTP. All four patients had lacking or strongly reduced vWF-cleaving protease activity. In another patient with chronic relapsing TTP, the protease deficiency was due to the presence in the patient plasma of an inhibitor that was found to be an IgG. We conclude that constitutional as well as acquired deficiency of vWF-cleaving protease may predispose to clinical manifestation of TTP.
Authors:
M Furlan; B Lämmle
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Baillière's clinical haematology     Volume:  11     ISSN:  0950-3536     ISO Abbreviation:  Baillieres Clin. Haematol.     Publication Date:  1998 Jun 
Date Detail:
Created Date:  1999-06-15     Completed Date:  1999-06-15     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8800474     Medline TA:  Baillieres Clin Haematol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  509-14     Citation Subset:  IM    
Affiliation:
Central Haematology Laboratory, University Hospital, Bern, Switzerland.
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MeSH Terms
Descriptor/Qualifier:
ADAM Proteins
Humans
Male
Metalloendopeptidases / deficiency*
Purpura, Thrombotic Thrombocytopenic / blood*,  genetics
von Willebrand Factor / metabolism*
Chemical
Reg. No./Substance:
0/von Willebrand Factor; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human; EC 3.4.24.-/Metalloendopeptidases

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