Download PDF
Thromb Haemost 1976; 35(02): 289-294
DOI: 10.1055/s-0038-1647921
Original Article
Schattauer GmbH

Molecular Variant of Factor VII

Ernest Briët
1   Hemostasis and Thrombosis Research Unit (Div. of Hematology, Dept. of Medicine) University Hospital Leiden, The Netherlands.
,
Emil A. Loeliger
1   Hemostasis and Thrombosis Research Unit (Div. of Hematology, Dept. of Medicine) University Hospital Leiden, The Netherlands.
,
Nico H. van Tilburg
1   Hemostasis and Thrombosis Research Unit (Div. of Hematology, Dept. of Medicine) University Hospital Leiden, The Netherlands.
,
Jan J. Veltkamp
1   Hemostasis and Thrombosis Research Unit (Div. of Hematology, Dept. of Medicine) University Hospital Leiden, The Netherlands.
› Author Affiliations
Further Information

Publication History

Received 26 March 1975

Accepted 03 September 1975

Publication Date:
02 July 2018 (online)

Also available at
    Permissions and Reprints

Summary

Two patients with severe factor VII deficiency appeared to be different with respect to the presence of inactive factor VH-like material in their plasma.

The inactive material, demonstrated in one patient’s plasma and adsorbable onto BaSO4, inhibits the thrombotest-system and is capable of neutralizing a heterologous antibody against factor VII in a fluid phase system.

 

  • References

  • 1 Brüning P. F, Loeliger E. A. 1971; Prothrombal: A new concentrate of human prothrombin complex for clinical use. British Journal of Haematology 21: 377.
    CrossrefPubMedSearch in Google Scholar
  • 2 Cleton F. J, Loeliger E. A. 1960; Two typical hereditary charts of congenital factor VII deficiency. Thrombosis et Diathesis Haemorrhagica 5: 87.
    PubMedSearch in Google Scholar
  • 3 Denson K. W. E, Biggs R, Mannucci P. M. 1968; An investigation of three patients with Christmas disease due to an abnormal type of factor IX. Journal of Clinical Pathology 21: 160.
    CrossrefPubMedSearch in Google Scholar
  • 4 Fantl P, Sawers R. J, Marr A. G. 1956; Investigation of a haemorrhagic disease due to beta-prothromboplastin deficiency complicated by a specific inhibitor of thromboplastin formation. Australasian Annals of Medicine 5: 163.
    CrossrefPubMedSearch in Google Scholar
  • 5 Girolami A, Brunetti A, De Marco L, Fioretti D. 1974; Dilution curve studies in prothrombin complex factors deficiencies and abnormalities. Blut 29: 134.
    CrossrefPubMedSearch in Google Scholar
  • 6 Girolami A, Molaro G, Løzzarin M, Scarpa R, Brunetti A. 1970; Congenital haemorrhagic condition similar but not identical to factor X deficiency. Haemorrhagic state due to an abnormal factor X?. Scandinavian Journal of Haematology 7: 91.
    PubMedSearch in Google Scholar
  • 7 Girolami A, Muller A. D, Hemker H. C. 1972; Lack of PIVKA effect in the abnormal factor X (factor X Friuli) coagulation disorder. Haemostasis 1: 23.
    PubMedSearch in Google Scholar
  • 8 Goodnight S. H, Feinstein D. I, Østerud B, Rapaport S. I. 1971; Factor VII antibody-neutralizing material in hereditary and acquired factor VTI deficiency. Blood 38: 1.
    PubMedSearch in Google Scholar
  • 9 Graham J. B, Barrett D. A, Blombäck B, Cann H. M, Hardisty R. M, Larrieu M. J, Renwick J. H. 1973; Reports from the international committee of haemostasis and thrombosis. I. A genetic nomenclature for human blood coagulation. Thrombosis et Diathesis Haemorrhagica 30: 2.
    PubMedSearch in Google Scholar
  • 10 Hemker H. C, Muller A. D. 1968; Kinetic aspects of the interaction of blood-clotting enzymes. Thrombosis et Diathesis Haemorrhagica 20: 78.
    PubMedSearch in Google Scholar
  • 11 Hemker H. C, Swart A. C. W, Alink A. J. M. 1972; Artificial reagents for factor VII and factor X, a computer programme for obtaining reference tables for one-stage determinations in the extrinsic system. Thrombosis et Diathesis Haemorrhagica 27: 205.
    PubMedSearch in Google Scholar
  • 12 Hemker H. C, Veltkamp J. J, Hensen A, Loeliger E. A. 1963; Nature of prothrombin biosynthesis: Preprothrombinaemia in vitamin K-deficiency. Nature 200: 589.
    CrossrefPubMedSearch in Google Scholar
  • 13 Imperato DI. C, Dettori A. G. 1958; Ipoflbrinogenemia conginita con fibrinoastenia. Helvetica Paediatrica Acta 13: 380.
    PubMedSearch in Google Scholar
  • 14 Roller F, Loeliger E. A, Duckert F. 1951; Experiments on a new clotting factor (factor VII). Acta Haematologica 6: 1.
    CrossrefPubMedSearch in Google Scholar
  • 15 Levanon M, Rimon S, Shani M, Ramot B, Goldberg E. 1972; Active and inactive factor VII in Dubin-Johnson syndrome with factor VII deficiency, hereditary factor VTI deficiency and on coumarin administration. British Journal of Haematology 23: 669.
    CrossrefPubMedSearch in Google Scholar
  • 16 Meyer D, Bidwell E, Larrieu M. J. 1972; Cross-reacting material in genetic variants of haemophilia B. Journal of Clinical Pathology 25: 433.
    CrossrefPubMedSearch in Google Scholar
  • 17 Roberts H. R, Grizzle J. E, McLester W. D, Penick G. D. 1968; Genetic variants of hemophilia B: Detection by means of a specific PTC inhibitor. Journal of Clinical Investigation 47: 360.
    CrossrefPubMedSearch in Google Scholar
  • 18 Shapiro S. S, Martinez J, Holburn R. R. 1969; Congenital dysprothrombinemia, an inherited structural disorder of human prothrombin. Journal of Clinical Investigation 48: 2251.
    CrossrefPubMedSearch in Google Scholar
  • 19 Zimmerman T. S, Edgington T. S. 1974; Molecular immunology of factor VIII. Annual Review of Medicine 25: 303.
    CrossrefPubMedSearch in Google Scholar