Alterations of coagulation and fibrinolysis in patients with angioedema due to C1-inhibitor deficiency
Publication year
2012Source
Clinical and Experimental Immunology, 167, 3, (2012), pp. 472-8ISSN
Publication type
Article / Letter to editor
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Organization
Laboratory of Hematology
Journal title
Clinical and Experimental Immunology
Volume
vol. 167
Issue
iss. 3
Page start
p. 472
Page end
p. 8
Subject
NCEBP 14: Cardiovascular diseasesAbstract
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.
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- Academic publications [238441]
- Faculty of Medical Sciences [90373]
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