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Thromb Haemost 1961; 05(02): 187-200
DOI: 10.1055/s-0038-1654919
Originalarbeiten – Original Article – Travaux Originaux
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Coagulation Studies in a Case of Hageman Trait

E. A Loeliger
1   Clinic for Internal Medicine (Director: Prof. J. Mulder, M.D.) University Hospital, Leyden, The Netherlands
,
A Hensen
1   Clinic for Internal Medicine (Director: Prof. J. Mulder, M.D.) University Hospital, Leyden, The Netherlands
› Author Affiliations
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Publication Date:
21 June 2018 (online)

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Summary

After a brief review of the data concerning the cases of Hageman trait hitherto reported in the literature, a patient with severe Hageman factor (HF) “deficiency” (HF-activity below 0.05% of normal) is described. The patient has no haemorrhagic diathesis.

In vitro, intrinsic coagulability and fibrinolytic activity are grossly disturbed. On the basis of a diminished fibrinolytic activity in vivo, the rather high activity of several coagulation factors in patient’s plasma could be explained.

Thromboplastin formation, once initiated, is normal as to rate; the amount of thromboplastin formed is possibly slightly diminished. Prothrombin consumption is normal. These findings are in agreement with a normal rate ot clot formation, as measured by means of thrombelastography.

Normal HF seems to be an initiator of blood coagulation only and not an activator or a substrate involved in thromboplastin formation.

The weak anticoagulant property of Hageman trait plasma, described by several authors, is not necessarily due to an inhibitor; it can be explained by the assumption that, in Hageman trait, HF shows normal glass adsorbability and only a very deficient glass activation.

 

  • References

  • 1 Bangham A. D, Pethica B. A, Seaman G. VF. The charged groups at the interface of some blood cells. Biochem. J. 69: 12-19 1958;
    CrossrefPubMedSearch in Google Scholar
  • 2 Caen J, Bernard J. Deficit en facteur Hageman. Rev. Hémat. 13: 154-162 1958;
    PubMedSearch in Google Scholar
  • 3 Frick P. G, Hagen P. S. Severe coagulation defect without hemorrhagic symptoms caused by a deficiency of the fifth plasma thromboplastin precursor. J. Lab. clin. Med. 47: 592-601 1956;
    PubMedSearch in Google Scholar
  • 4 Geiger M, Duckert F, Koller F. Quantitative Bestimmungen von Faktor VIII (antihämophilem Globulin) und Faktor IX (Christmas-Faktor, PTC) bei Blutersippen. 5. Kongr. Eur. Gesellsch. Hämatol. 1956; 413.
    PubMedSearch in Google Scholar
  • 5 Hardisty R. M, Margolis J. The role of Hageman factor in the initiation of blood coagulation. Brit. J. Haemat. 05: 203-211 1959;
    CrossrefPubMedSearch in Google Scholar
  • 6 Jim R. T. S, Goldfein S. Hageman Trait (Hageman Factor Deficiency. Amer. J. Med. 23: 824-831 1957;
    CrossrefPubMedSearch in Google Scholar
  • 7 Johnston C. L, Ferguson J. H, O’Hanlon F. A. Surface activation of plasma clotting: a function of Hageman factor. Proc. Soc. exp. Biol. (N. Y) 99: 197-201 1958;
    CrossrefPubMedSearch in Google Scholar
  • 8 Larrieu M. J, Soulier J. P, Culot Y. Deficit en facteur Hageman. Sang. 28: 152-159 1957;
    PubMedSearch in Google Scholar
  • 9 Loeliger E. A, Hers P. hJF. Chronic antithrombinaemia (antithrombin V) with haemorrhagic diathesis in a case of rheumatoid arthritis with hypergammaglobulin-aemia. Thromb. Diath. haem. 01: 499 1957;
    PubMedSearch in Google Scholar
  • 10 Loeliger E. A. Prothrombin as Co-factor of the circulating anticoagulant in systemic lupus erythematosus? Thromb. Diath. haem. 03: 237 1959;
    PubMedSearch in Google Scholar
  • 11 Margolis J. Initiation of blood coagulation by glass and related surfaces. J. Physiol. 137: 95-109 1957;
    CrossrefPubMedSearch in Google Scholar
  • 12 Margolis J. Hageman factor in plasma foreign surface reactions. Nature (Lond) 182i: 1102-1103 1958;
    PubMedSearch in Google Scholar
  • 13 Margolius A, Ratnoff O. D. Observations on the hereditary nature of Hageman trait. Blood 11: 565-569 1956;
    PubMedSearch in Google Scholar
  • 14 Niewiarowski S, Prou-Wartelle O. Rôle du facteur contact (facteur Hageman) dans la fibrinolyse. Thromb. Diath. haem. 03: 593 1959;
    PubMedSearch in Google Scholar
  • 15 Ramot B, Singer K, Heller P, Zimmerman H. J. Hageman factor (HF) deficiency. Blood 11: 745 1956;
    PubMedSearch in Google Scholar
  • 16 Rapaport S. I. Evidence that glass increases plasma PTA activity. J. Lab. clin. Med. 52: 624-633 1958;
    PubMedSearch in Google Scholar
  • 17 Ratnoff O. D. A familial trait characterized by deficiency of a clot-promoting fraction of plasma. J. Lab. clin. Med. 44: 915 1954;
    PubMedSearch in Google Scholar
  • 18 Ratnoff O. D, Colopy J. E. A familial hemorrhagic trait associated with a deficiency of a clot promoting fraction of plasma. J. clin. Invest 34: 602 1955;
    CrossrefPubMedSearch in Google Scholar
  • 19 Ratnoff O. D, Margolius A. Hageman trait: an asymptomatic disorder of blood coagulation. Trans. Ass. Amer. Physiol. 68: 149-154 1955;
    PubMedSearch in Google Scholar
  • 20 Ratnoff O. D, Rosenblum J. M. The role of Hageman factor in the initiation of clotting by glass. J. Lab. clin. Med. 47: 592 1956;
    PubMedSearch in Google Scholar
  • 21 Sjolin K. E. Deficiency of the Hageman factor demonstrated by the thrombin generation test. Thromb. Diath. haem. 01: 153 1957;
    PubMedSearch in Google Scholar
  • 22 Soulier J. P, Larrieu M. J. Deficit en 3ème factor prothromboplastique plasmatique. Rapports entre le PTA et le facteur Hageman. Thromb. Diath. haem. 02: 1 1958;
    PubMedSearch in Google Scholar
  • 23 Soulier J. P, Wartelle O, Ménaché D. Hageman trait and PTA deficiency; the role of contact of blood with glass. Brit. J. Haemat. 05: 121 1959;
    CrossrefPubMedSearch in Google Scholar
  • 24 Vroman L. Surface contact and thromboplastin formation. Thesis, Utrecht (Holland). 1958
    PubMedSearch in Google Scholar
  • 25 Waaler B. A. Contact activation in the intrinsic blood clotting system. Supplement to Vol. 11 (1959) of the Scand. J. clin. Lab. Invest..
    PubMed
  • 26 Wright I. S, Koller F, Streuli F. New blood clotting factors. Thromb. Diath. haem. Suppl. ad. Vol. IV. 1960
    PubMedSearch in Google Scholar