Comprehensive study into the activation of the plasma enzyme systems during attacks of hereditary angioedema due to C1-inhibitor deficiency

Dorottya Csuka, Nóra Veszeli, Éva Imreh, Zsuzsanna Zotter, Judit Skopál, Zoltán Prohászka, Lilian Varga, Henriette Farkas

Research output: Contribution to journalArticle

23 Citations (Scopus)


Background: The activation of plasma enzyme systems contributes to hereditary angioedema attacks. We aimed to study the activation markers of the fibrinolytic, coagulation, and contact systems in a larger number of paired samples obtained from the same C1-INH-HAE patients in symptom-free periods and during attacks. Methods: Eleven parameters (Factors XI, XII, and C1-inhibitor activity; the concentrations of the D-dimer, prothrombin fragments 1∈+∈2, plasminogen, plasminogen activator inhibitor-1 [PAI-1], thrombin-anti-thrombin III [TAT] complex, fibrinogen) were measured along with prothrombin time and activated partial thromboplastin time (aPTT), using commercial kits. We compared these markers in samples obtained from the same 39 patients during attack-free periods and during 62 edematous episodes. Forty healthy subjects of matching sex and age served as controls. Results: Compared with the healthy controls, significantly higher FXI and FXII activity (p∈=∈0.0007, p∈=∈0.005), as well as D-dimer (p∈<∈0.0001), prothrombin fragments 1∈+∈2 (p∈<∈0.0001), and TAT (p∈=∈0.0303) levels were ascertained in the patients during symptom-free periods. The evaluation of samples from symptom-free periods or obtained during attacks revealed the increase of FXII activity, as well as of the concentration of D-dimer, prothrombin fragments 1∈+∈2, and TAT during edematous episodes. PAI-1 level, prothrombin time, and aPTT decreased significantly during attacks, compared with symptom-free periods. D-dimer level was significantly higher during multiple- vs. single-site attacks. Conclusions: Comparing a large number of paired samples from symptom-free periods or from edematous episodes allowed accurate appraisal of the changes occurring during attacks. Moreover, our study pointed out that individual episodes may be characterized by different marker patterns.

Original languageEnglish
Article number132
JournalOrphanet journal of rare diseases
Issue number1
Publication statusPublished - Oct 9 2015


  • Attack location
  • C1-inhibitor
  • Edematous attack
  • Hereditary angioedema
  • Plasma enzyme systems

ASJC Scopus subject areas

  • Genetics(clinical)
  • Pharmacology (medical)

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