Download PDF
Thromb Haemost 1965; 14(03/04): 346-360
DOI: 10.1055/s-0038-1654874
Originalarbeiten — Original Articles — Travaux Originaux
Schattauer GmbH

A Congenital Inhibitor against Factor IX

H STOCKER
1   Department of Internal Medicine, University of Basle (Head: Prof. Dr. F. Koller) (Work instructed by Dr. F.Duckert)
› Author Affiliations
Further Information

Publication History

Publication Date:
27 June 2018 (online)

Also available at
    Permissions and Reprints

Summary

A new congenital hemorrhagic diathesis, which is due to the presence of a circulating inhibitor against factor IX, simulating a hemophilia B, is described. It is demonstrated, that the inhibitor becomes much less efficient when the factor VUI-concentration decreases. A hypothesis is given which should explain the role of factor VIII in the inhibition process.

 

  • References

  • 1 Bachmann F, Duckert F, Koller F. The Stuart-Prower Factor Assay and Its Clinical Significance. Thrombos. Diathes, haemorrh. (Stuttg.) 02: 24 1958;
    PubMedGoogle Scholar
  • 2 Baker S. J, Jacob E. A Hemorrhagic Disorder in Pregnancy Due to an “anticoagulant” Preventing the Conversion of Fibrinogen to Fibrin. J. clin. Path. 13: 214 1960;
    CrossrefPubMedGoogle Scholar
  • 3 Beck E, Bachmann F, Muller J, Duckert F. Formation of an Inhibitor against Factor VIII as a Postoperative Complication in a severe Case of Hemophilia A. Thrombos. Diathes. haemorrh. (Stuttg.) 07: 27 1962;
    PubMedGoogle Scholar
  • 4 Butler R, Röhrig S, Siegenthaler P, Moeschlin S. A special Hemophilic Syndrome. Acta haemat. (Basel.) 22: 292 1959;
    CrossrefPubMedGoogle Scholar
  • 5 Clauss A. Gerinnungsphysiologische Schnellmethode zur Bestimmung des Fibrinogens. Acta haemat. (Basel.) 17: 4 1957;
    PubMedGoogle Scholar
  • 6 Santa RDeila. Présence d’un anticoagulant dans le sang d’une malade atteinte de lupus érythémateux. Schweiz, med. Wschr. 89: 1031 1959;
    PubMedGoogle Scholar
  • 7 Douglas A. S. An Atypical Circulating Thromboplastin Inhibitor. Brit. J. Haemat. 04: 302 1958;
    CrossrefPubMedGoogle Scholar
  • 8 Duckert F, Flückiger P, Isenschmid H, Matter M, Vogel-Meng J, Koller F. A Modification of the Thromboplastin Generation Test. Acta haemat. (Basel.) 12: 197 1954;
    CrossrefPubMedGoogle Scholar
  • 9 Ferguson J. V, Johnston C. L, Howell D. A. Anti-AcG: Specific Circulating Inhibitor of the Labile Clotting Factor. Proc. Soc. exp. Biol. (N. Y.) 95: 567 1957;
    CrossrefPubMedGoogle Scholar
  • 10 Fisch U. Über einen neuen Accelerator der Blutthrombokinasebildung. Thrombos. Diathes. haemorrh. (Stuttg.) 02: 60 1958;
    PubMedGoogle Scholar
  • 11 Geiger M. Personal communication..
    PubMed
  • 12 Geiger M, Duckert F, Koller F. Quantitative Bestimmungen von Faktor VIII und IX bei Blutersippen. 413 5th. Cong. Europ. Soc. Haemat. Freiburg i.Br. 1956;
    PubMedGoogle Scholar
  • 13 Jung E. G, Duckert F, Frick P. G. Autoimmunes Antithromboplastin. Schweiz, med. Wschr. 91: 419 1961;
    PubMedGoogle Scholar
  • 14 Koller F. Vitamin K und seine klinische Bedeutung. Leipzig.. 1941
    PubMedGoogle Scholar
  • 15 Koller F, Loeliger A, Duckert F. Experiments on a new Clotting Factor. Factor VII. Acta haemat. (Basel.) 06: 1 1951;
    PubMedGoogle Scholar
  • 16 Loeliger A. Chronic Antithrombinaemia (Antithrombin V) with Hemorrhagic Diathesis in a Case of Rheumatoid Arthritis with Hypergammaglobulinaemia. Thrombos. Diathes. haemorrh. (Stuttg.) 01: 499 1957;
    PubMedGoogle Scholar
  • 17 Loeliger A. Prothrombin as Co-Factor of a Circulating Anticoagulant in Systemic Lupus Erythematosus ? Thrombos. Diathes. haemorrh. (Stuttg.) 03: 237 1959;
    PubMedGoogle Scholar
  • 18 Matter M. Personal communication..
    PubMed
  • 19 Medal L. S, Lisker R. Circulating Anticoagulants in Disseminated Lupus Erythematosus. Brit. J. Haemat. 05: 284 1959;
    CrossrefPubMedGoogle Scholar
  • 20 Nilsson I. M, Skanse B, Gydell K. Circulating Anticoagulant after Pregnancy and Its Response to ACTH. Acta haemat. (Basel.) 19: 40 1958;
    CrossrefPubMedGoogle Scholar
  • 21 Nussey A. M, Dawson D. W. Haemophilia-like Disease Due to an Auto-Antibody. Brit, med. J. 5053: 1077 1957;
    PubMedGoogle Scholar
  • 22 O’Brien J. R. Un inhibiteur de la maladie de Christmas. Rev. Hémat. 12: 294 1957;
    PubMedGoogle Scholar
  • 23 Perkins H. A, Acra D. J. The circulating Anticoagulant in Disseminated Lupus Erythematosus. Thrombos. Diathes. haemorrh.. (Stuttg.) 05: 250 1960;
    PubMedGoogle Scholar
  • 24 Post G. R, den Ottolander GJH, Hoorweg P. G. A Case of Hemorrhagic Diathesis Due to a Circulating Anticoagulant. Acta haemat. (Basel.) 18: 290 1957;
    CrossrefPubMedGoogle Scholar
  • 25 Straub W. Separation of Coagulation Factors by means of Starch Gel Electrophoresis. Thrombos. Diathes. haemorrh. (Stuttg.) 04: 451 1960;
    PubMedGoogle Scholar
  • 26 Yin E. T, Duckert F. Purification of Factor IX or Its Precursor. Isolation of a Hageman - PTA Fraction. Thrombos. Diathes. haemorrh. (Stuttg.) 06: 215 1961;
    PubMedGoogle Scholar
  • 27 Yin E. T, Duckert F. The Formation of Intermediate Product I in a Purified System. The Role of Factor IX or of its Precursor and of a Hageman - PTA Fraction. Thrombos. Diathes. haemorrh. (Stuttg.) 06: 224 1961;
    PubMedGoogle Scholar
  • 28 Zbinden J, Leupold R. Über Hemmkörperhämophilie. Schweiz, med. Wschr. 87: 1478 1957;
    PubMedGoogle Scholar
  • 29 Zollinger W. Familienuntersuchung bei congenitalem Faktor-VII-Mangel. Arch. KlausStiftung. Bd. 38, Heft. 1-2: 5 1963;
    PubMedGoogle Scholar