Document Detail

Factor V inhibitor associated with Sjögren's syndrome.
MedLine Citation:
PMID:  7772528     Owner:  NLM     Status:  MEDLINE    
We report an unusual case of a 74-year-old male who developed a serum autoantibody reactive with human coagulation factor V (FV) in an activated form, as demonstrated by coagulation studies and immunoblotting analysis. Despite marked prolongation of a prothrombin time and an activated partial thromboplastin time in this patient, the inhibitor was not associated with clinical bleeding but with multiple cerebral infarctions. The patient had suffered from Sjögren's syndrome with polyclonal hypergammaglobulinaemia. The patient's purified IgG, an immediately acting inhibitor to FV, reacted with a light chain of thrombin-activated FV (FVa) and inhibited the procoagulant activity of FVa without affecting the cleavage of FVa by activated protein C. The FV inhibitor may arise from activation of FV with consequent exposure of neoantigen during the activation of coagulation cascade in the patient with an autoimmune disorder for the background.
T Koyama; T Saito; T Kusano; S Hirosawa
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  British journal of haematology     Volume:  89     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  1995 Apr 
Date Detail:
Created Date:  1995-07-13     Completed Date:  1995-07-13     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  893-6     Citation Subset:  IM    
First Department of Internal Medicine, Tokyo Medical and Dental University, Japan.
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MeSH Terms
Blood Coagulation
Factor V / antagonists & inhibitors*
Immunoglobulin Light Chains / analysis
Partial Thromboplastin Time
Prothrombin Time
Sjogren's Syndrome / blood,  etiology*
Reg. No./Substance:
0/Immunoglobulin Light Chains; 9001-24-5/Factor V

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