Download PDF
Thromb Haemost 1995; 74(04): 1103-1106
DOI: 10.1055/s-0038-1649888
Original Article
Coagulation
Schattauer GmbH Stuttgart

Factor VIIa in Patients with C1-Inhibitor Deficiency

Erik Waage Nielsen
1   The Department of Anaesthesiology, Nordland Central Hospital, Bodø and University of Tromsø, Norway
,
James Morrissey
2   The Oklahoma Medical Research Foundation, Oklahoma City, USA
,
Jan Ole Olsen
3   The Institute of medical Biology, University of Tromsø, Norway
,
Bjarne Østerud
3   The Institute of medical Biology, University of Tromsø, Norway
› Author Affiliations
Further Information

Publication History

Received 08 February 1995

Accepted after resubmission 23 June 1995

Publication Date:
09 July 2018 (online)

  PDF Download  Permissions and Reprints

Summary

In hereditary angioedema (HAE), normal C1-inhibitor (CI-INH) is low and the contact system aetivated. Recently, the findings of a tissue factor mutant selectively deficient in promoting the conversion of FVII to FVIIa, but with retained cofactor for FVIIa, made it possible to examine reliably the pre-existing content of FVIIa in HAH patients. This was of interest as FXIIa (mainly inhibited by (CI-INH) is able to activate FVII directly. FVIIa in 21 remission HAH patients were within normal limits but nearly doubled as compared to their 23 normal siblings (p = 0.0017). Cold promoted activation of FVII (CPA) (common Clot assay) was displayed in plasma of all 5 untreated patients (CI-INH function <35%), but not in plasma of 2 patients treated prophylactically with danazol (CI-INH function about 40%). These results suggest that there is a minute, yet significant activation of FVII in patients with CI-INH deficiency.

 

  • References

  • 1 Pixley RA, Delacadena R, Page JD, Kaufman N, Wyshock EG, Cluing A, Taylor FB, Column RW. The eontael syslem eontrihules to hypoiension hul nol disseminated intravascular coagulation in lethal bacteremia in vivo use of a monoclonal anti-factor-XII antibody to block contact activation in baboons. J Clin Invest 1993; 91: 61-68
    CrossrefPubMedGoogle Scholar
  • 2 Burman JF, Chung HL, Lane DA, Philippou II, Adami A, Lincoln JC R. Role of factor XII in thrombin generation and fibrinolysis during cardiopulmonary bypass. Lancet 1994; 344: 1192-1193
    CrossrefPubMedGoogle Scholar
  • 3 Colman RW, Scott CP, Pixley RA, Cadcna RA. Dl Effect of heparin on the inhibition of the eontael system enzymes. Ann N Y Acad Sei 1989; 536: 95-103
    PubMedGoogle Scholar
  • 4 Adams J, Ridley CM. Hereditary angio-oedema with easy bruising. Br J Dermatol 1983; 91-92
    PubMedGoogle Scholar
  • 5 Mincshita M, Kayashima S, Makishima M, Kimura P, Sasaki T, Takagi S, Matuura Y, Shimomura S, Yamamoto K, Satou N. Hereditary angioneurotic edema associated with DIC and treated effectively by nalamostat mesilate (Japanese). Rinsho Ketsueki 1992; 33: 30-35
    PubMedGoogle Scholar
  • 6 RadclilTe R, Bagdasarian A, Colman R, Nemerson Y. Activation of bovine factor VII by hageman factor fragments. Blood 1977; 50: 611-617
    PubMedGoogle Scholar
  • 7 Seligsohn U, østerud B, Brown SF, Griffin JH, Rapaport SI. Activation of human factor VII in plasma and purified systems. J Clin Invest 1979; 64: 1056-1065
    CrossrefPubMedGoogle Scholar
  • 8 Morrissey JH, Macik BG, Neuenschwander PF, Comp PC. Quantitation of activated factor VII levels in plasma using a tissue factor mutant selectively deficient in promoting factor VII activation. Blood 1993; 81: 734-744
    PubMedGoogle Scholar
  • 9 Gjonnaess HA. Cold promoted activation of factor VII. I. Evidence for the existence of an activator Thromb Diath Haemorrh 1972; 28: 155-168
    PubMedGoogle Scholar
  • 10 Van Royen EA, Lohmann S, Voss M, Pondmann KW. Cl inactivator and cold-promoted activation of factor VII. J Lab Clin Med 1978; 92: 152-163
    PubMedGoogle Scholar
  • 11 Donaldson VH. Cold enhancement of blood coagulation: observation on the role of Cl-inhibitor. Acta path microbiol immunol scand 1984; 92: 41-47
    PubMedGoogle Scholar
  • 12 Czendlik C, Lammle B, Duckert F. Cold promoted activation and factor XII, prekallikrein and Cl-inhibitor. Thromb Haemost 1985; 242-244
    Article in Thieme ConnectPubMedGoogle Scholar
  • 13 Patston PA, Gettins P, Beechem J, Schapira M. Mechanism of serpin action: Evidence that Cl inhibitor functions as a suicide substrate. Biochemistry 1991; 30: 8876-8882
    CrossrefPubMedGoogle Scholar
  • 14 Østerud B, Rapaport SI. Synthesis of intrinsic factor X activator. Inhibition of the function of formed activator by antibodies to factor VIII and to factor IX Biochemistry 1970; 9: 1854-1861
    CrossrefPubMedGoogle Scholar
  • 15 Munkvad S, Jespersen J, Gram J, Overgaard K, Ranby M. Effects of methylamine and heparin on a rapid chromogenic assay Cl-esterase inhibitor in plasma. Clin Chem 1990; 36: 737-741
    PubMedGoogle Scholar
  • 16 Cugno M, Nijens J, Hack E, Eerenberg A, Frangi D, Agostoni A, Cicardi M. Plasma levels of Cl-inhibitor complexes and cleaved Cl-inhibitor in patients with hereditary angioneurotic edema. J Clin Invest 1990; 85: 1215-1220
    CrossrefPubMedGoogle Scholar
  • 17 Cugno M, Hack CE, Deboer JP, Eerenberg AJ M, Agostoni A, Cicardi M. Generation of plasmin during acute attacks of hereditary angioedema. J Lab Clin Med 1993; 121: 38-43
    PubMedGoogle Scholar
  • 18 Schapira M, Silver LD, Scott CF, Schmaier AH, Prograis LJ, Curd JG, Colman RW. Prekallikrein activation and high-molecular-weight kininogen consumption in hereditary angioedema. N Engl J Med 1983; 308: 1050-1054
    CrossrefPubMedGoogle Scholar
  • 19 Chibber G, Cohen A, Lane S, Farber A, Meloni FJ, Schmaier AH. Immunoblotting of plasma in a pregnant patient with hereditary angioedema. J Lab Clin Med 1990; 115: 112-121
    PubMedGoogle Scholar
  • 20 Østerud B, Laake K, Prydz H. The activation of human factor IX. Thromb Diath Haemorrh 1975; 33: 553-563
    PubMedGoogle Scholar
  • 21 Mitropoulos KA, Reeves BE A, Obrien DP, Cooper JA, Martin JC. The Relationship between factor-VII coagulant activity and factor-XII-activation induced in plasma by endogenous or exogenously added contact surface. blood Coagulant Fibrinol 1993; 4: 223-234
    PubMedGoogle Scholar
  • 22 Pixley RA, Schapira M, Colman RW. The regulation of human factor Xlla by plasma proteinase inhibitors. J Biol Chem 1985; 260: 1723-1729
    PubMedGoogle Scholar
  • 23 Perkins W, Stables GI, Lever RS. Protein S Deficiency in lupus erythematosus secondary to hereditary angio-oedema. Br J Dermatol 1994; 130: 381-384
    CrossrefPubMedGoogle Scholar
  • 24 Dahlbäck B. Protein S and C4b-binding protein: Components involved inthe regulation of the protein C anticoagulant system. Thromb Haemost 1991; 66: 49-61
    Article in Thieme ConnectPubMedGoogle Scholar
  • 25 Donaldson VH, Rosen FS. Hereditary angioneurotic edema: A clinical survey. Pediatrics 1966; 37: 1017-1027
    PubMedGoogle Scholar
  • 26 Feller EJ, Spiro HM, Katz LA. Hereditary angioneurotic oedema: an unusual cause of recurring abdominal pain. Gut 1970; 11: 983-988
    CrossrefPubMedGoogle Scholar
  • 27 Donaldson VH. Cl activation in hereditary angioneurotic edema plasma: role of urokinase and inhibitors. J Appl Physiol 1968; 25: 416-424
    CrossrefPubMedGoogle Scholar
  • 28 Nilsson T, Bäck O. Elevated plasmin-alfa-2-antiplasmin complex levels in hereditary angioedema: evidence for the in vivo efficiency of the intrinsic fibrinolytic system. Thromb Res 1985; 40: 817-821
    CrossrefPubMedGoogle Scholar
  • 29 Ichinose A, Fujikawa K, Suyama T. The activation of pro-urokinase by plasma kallikrein and its inactivation by thrombin. J Biol Chem 1986; 261: 1253-1258
    PubMedGoogle Scholar
  • 30 Cameron CL, Fisslthaler B, Sherman A, Reddigari S, Silverberg M. Studies on contact activation: effects of surface and inhibitors. Med Prog Technol 1989; 15: 53-62
    PubMedGoogle Scholar
  • 31 Nielsen EW, Johansen HT, Straume B, Mollnes TE. Effect of time, temperature and additives on a functional assay of Cl inhibitor. J Immunol Methods 1994; 173: 245-251
    CrossrefPubMedGoogle Scholar
  • 32 Späth PJ, Wuthrich B, Butler R. Quantification of C1-inhibitor functional activities by immunodiffusion assay in plasma on patients with hereditary angioedema evidence of a functionally critical level of Cl-inhibitor concentration. Complement 1984; 1: 147-159
    CrossrefPubMedGoogle Scholar
  • 33 Ziccardi RJ. A new role for Cl-inhibitor in homeostasis: control of activation of the first component of human complement. J Immunol 1982; 128: 2505-2508
    PubMedGoogle Scholar