Document Detail

Alterations of coagulation and fibrinolysis in patients with angioedema due to C1-inhibitor deficiency.
MedLine Citation:
PMID:  22288590     Owner:  NLM     Status:  MEDLINE    
Patients with functional deficiency of C1-inhibitor (C1-INH) suffer from recurrent acute attacks (AA) of localized oedema associated with activation of the contact system, complement and fibrinolysis. To unravel further the role of coagulation and fibrinolysis in the pathophysiology of C1-INH deficiency, we performed simultaneous thrombin and plasmin generation measurements in plasma from patients with hereditary angioedema (HAE) due to C1-INH deficiency during AA (n = 23), in remission (R) (n = 20) and in controls (n = 20). During AA thrombin generation after in-vitro activation of plasma was higher than in controls, as demonstrated by shorter thrombin peak-time (P < 0·05), higher thrombin peak-height (P < 0·001) and increased area under the curve (AUC) (P < 0·05). Additionally, elevated levels of prothrombin fragment 1+2 (P < 0·0001) were observed in non-activated plasma from the same patients. In contrast, in activated plasma from patients during AA plasmin generation estimated as plasmin peak-height (P < 0·05) and plasmin potential (P < 0·05) was reduced, but non-activated plasma of the same patients showed elevated plasmin-anti-plasmin (PAP) complexes (P < 0·001). This apparent discrepancy can be reconciled by elevated soluble thrombomodulin (sTM) (P < 0·01) and thrombin activatable fibrinolysis inhibitor (TAFI) in patients during AA providing possible evidence for a regulatory effect on fibrinolysis. Plasminogen activator inhibitor-1 (PAI-1) was reduced in patients during AA indicating, together with the observed reduction of plasmin generation, the consumption of fibrinolytic factors. In conclusion, our results support the involvement of coagulation and fibrinolysis in the pathophysiology of HAE and show the possible application of simultaneous measurement of thrombin and plasmin generation to evaluate different clinical conditions in HAE patients.
M van Geffen; M Cugno; P Lap; A Loof; M Cicardi; W van Heerde
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical and experimental immunology     Volume:  167     ISSN:  1365-2249     ISO Abbreviation:  Clin. Exp. Immunol.     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-01-31     Completed Date:  2012-03-22     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  0057202     Medline TA:  Clin Exp Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  472-8     Citation Subset:  IM    
Copyright Information:
© 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology.
Laboratory of Hematology, Department of Laboratory Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands.
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MeSH Terms
Acute Disease
Aged, 80 and over
Blood Coagulation*
Case-Control Studies
Complement C1 Inhibitor Protein / metabolism*
Fibrinolysin / biosynthesis
Hereditary Angioedema Types I and II / blood*,  etiology*
Middle Aged
Thrombin / biosynthesis
Young Adult
Reg. No./Substance:
0/Complement C1 Inhibitor Protein; EC; EC

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