Document Detail


Acquired von Willebrand's disease: demonstration of a circulating inhibitor to the factor VIII complex in four cases.
MedLine Citation:
PMID:  6405778     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We have studied four patients suffering from acquired von Willebrand's disease. All patients had a severe bleeding diathesis with recurrent life-threatening haemorrhages. Three of the patients had a monoclonal gammopathy and one of these developed multiple myeloma. In three patients tested, a plasma inhibitor to ristocetin cofactor activity was detected. In each case this was localized to the IgG fraction of plasma. In addition, VIII:C activity was found to be associated with the IgG fraction of patients' plasma and altered mobility of VIII:C was detected on Laurell immunoelectrophoresis. Furthermore, plasma from all four patients and the IgG fraction therefrom resulted in a dissociation of normal VIII:C into two components separable by gel-filtration on Sepharose 6B. Finally the circulating half-life of the three factor VIII activities was found to be markedly reduced in the patients with acquired von Willebrand's disease. We conclude that in the patients studied the coagulation defect was related to the presence of a circulating inhibitor to the factor VIII complex and that this inhibitor was associated with the IgG fraction of plasma.
Authors:
B M Sampson; M Greaves; R G Malia; F E Preston
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  British journal of haematology     Volume:  54     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  1983 Jun 
Date Detail:
Created Date:  1983-07-15     Completed Date:  1983-07-15     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  233-44     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Aged
Antigens / metabolism
Chromatography, Gel
Factor VIII / antagonists & inhibitors*,  immunology,  metabolism
Female
Half-Life
Humans
Immunoglobulin G / metabolism
Male
Middle Aged
Platelet Aggregation / drug effects
Ristocetin / pharmacology
von Willebrand Diseases / blood*,  immunology
von Willebrand Factor
Chemical
Reg. No./Substance:
0/Antigens; 0/Immunoglobulin G; 0/von Willebrand Factor; 1404-55-3/Ristocetin; 9001-27-8/Factor VIII

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


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