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Thromb Haemost 1970; 23(03): 423-431
DOI: 10.1055/s-0038-1654068
Originalarbeiten – Original Articles – Travaux Originaux
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Absent Plasma Second Phase Platelet Aggregation Factor in a Patient with Hageman Trait[*]

P Okonkwo
1   Coagulation Section of the Circulation Laboratory, Boston, City Hospital and the Department of Medicine, Tufts University School of Medicine, Boston, Massachusetts
,
R Post
1   Coagulation Section of the Circulation Laboratory, Boston, City Hospital and the Department of Medicine, Tufts University School of Medicine, Boston, Massachusetts
,
H Sise
1   Coagulation Section of the Circulation Laboratory, Boston, City Hospital and the Department of Medicine, Tufts University School of Medicine, Boston, Massachusetts
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Publication Date:
27 June 2018 (online)

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Summary

A case is described of a patient with factor XII deficiency in whom the second phase of platelet aggregation was missing after addition of ADP to platelet rich plasma. There was very little aggregation on addition of epinephrine, collagen or thrombin. Clot retraction was absent, but all other platelet functions were normal and there was a very minor bleeding tendency. The defect was corrected by normal platelet poor plasma and was found to be due to a missing cofactor of platelet aggregation normally found in fraction 1–0. The defect could be distinguished from that seen in subjects ingesting aspirin as well as from the defect seen in patients with a long bleeding time, defective platelet aggregation with ADP, epinephrine and collagen at low concentrations, no correction by addition of normal platelet poor plasma, and a history of mild bleeding disorder. It is thought that this defect may exist in otherwise normal individuals and that attention in this case was called to it by the presence of the deficiency of factor XII. It could be conclusively shown that factor XII is not necessary for normal platelet aggregation.

* Supported by USPHS grant HE 10056.


 

  • References

  • 1 Sharp A. A. Viscous metamorphosis of blood platelets: A study of the relationship to coagulation factors and fibrin formation. Brit. J. Haemat 4: 28 1958;
    CrossrefPubMedGoogle Scholar
  • 2 Biggs R, Sharp A. A, Hardisty R. B, Stewart J, Davidson W. M. Defects in the early stages of blood coagulation: A report of four cases. Brit. J. Haemat 4: 177 1958;
    CrossrefPubMedGoogle Scholar
  • 3 Fantl P, Sweet B. Aggregation and activation of human blood platelets. Aust. J. exp. Biol. med. Sci 44: 123 1966;
    PubMedGoogle Scholar
  • 4 Sinakos Z, Sultan Y, Caen J, Bernard J. Le comportement des plaquettes dans les maladies hémorragiques. Presse méd 75: 269 1967;
    PubMedGoogle Scholar
  • 5 Jurgens J. The significance of the Hageman factor for the effect of wetable surface on thrombocytes. Thrombos. Diathes. hemorrh. (Stuttg.) 7: 48 1962;
    PubMedGoogle Scholar
  • 6 Iatridis P. G, Ferguson J. H, Iatridis S. G. Surface factor mechanisms in relation to blood platelets: Evidence that activated Hageman factor is present on the surface of platelets. Thrombos. Diathes. haemorrh. (Stuttg.) 11: 355 1964;
    PubMedGoogle Scholar
  • 7 Horowitz H. I, Fujimoto M. M. Association of factors XI and XII with blood platelets. Proc. Soc. exper. Biol. (N. Y.) 119: 487 1965;
    CrossrefPubMedGoogle Scholar
  • 8 Barth P, Pfleiderer B, Kommerell Th. The effect of the contact factors of the coagulation system on platelet aggregation. Thrombos. Diathes. haemorrh. (Stuttg.) 16: 378 1966;
    PubMedGoogle Scholar
  • 9 Nossel H. L, Wilner G. D, LeRoy E. C. Importances of polar groups for initiating blood coagulation and aggregating platelets. Nature (Lond.) 221: 75 1969;
    CrossrefPubMedGoogle Scholar
  • 10 Hardisty R. M, Hutton R. A. Bleeding tendency associated with “new” abnormality of platelet behaviour. Lancet I: 983 1967;
    PubMedGoogle Scholar
  • 11 Sahud M. A, Aggeler P. M. Platelet dysfunction-differentiation of a newly recognized primary type from that produced by aspirin. New Engl. J. Med 280: 453 1969;
    CrossrefPubMedGoogle Scholar
  • 12 Weiss H. J, Ghervenick P. A, Zalusky R, Factor A. A familial defect in platelet function associated with impaired release of adenosine diphosphate. New Engl. J. Med 281: 1264 1969;
    CrossrefPubMedGoogle Scholar
  • 13 Sherman L. A, Goldstein M. A, Sise H. S. Circulating anticoagulant (antifactor VIII) treated with immunosuppressive drugs. Thrombos. Diathes. haemorrh. (Stuttg.) 21: 249 1969;
    PubMedGoogle Scholar
  • 14 Rand J. J, Moloney W. G, Sise H. S. Coagulation defects in acute promyelocytic leukemia. Arch, intern. Med 123: 39 1969;
    CrossrefPubMedGoogle Scholar
  • 15 Salzman E. W. Measurement of platelet adhesiveness. A simple in vitro technique demonstrating an abnormality in von Willebrand’s disease. J. Lab. clin. Med 62: 724 1963;
    PubMedGoogle Scholar
  • 16 Brecher G, Cronkite E. P. Estimation of the number of platelets by phase microscopy. In: The Coagulation of Blood, Methods of Study. 41 Grune & Stratton, Inc.; N. Y.: 1955
    Google Scholar
  • 17 Nierowski S, Thomas D. Platelet aggregation by ADP and thrombin. Nature (Lond.) 212: 1544 1966;
    CrossrefPubMedGoogle Scholar
  • 18 Born G. V. R. Quantitative investigations into aggregation of blood platelets. J. Physiol 262: 67-68 1962;
    PubMedGoogle Scholar
  • 19 Weiss H. J, Eichelberger J. W. The detection of platelet defects in patients with mild bleeding disorders. Use of a quantitative assay for platelet factor 3. Amer. J. Med 32: 872 1962;
    CrossrefPubMedGoogle Scholar
  • 20 Spaet T. H, Cintron J. Studies on platelet factor 3 availability. Brit. J. Haemat 11: 269 1965;
    CrossrefPubMedGoogle Scholar
  • 21 Weiss H. J. Platelet aggregation, adhesion, and adenosine diphosphate release in thrombo-pathia (platelet factor 3 deficiency). Amer. J. Med 43: 570 1967;
    CrossrefPubMedGoogle Scholar
  • 22 Blombäck B, Blombäck M. Purification of human and bovine fibrinogen. Arkiv. for Kemi 20: 423 1956;
    PubMedGoogle Scholar
  • 23 Aster R. H, Jandl J. H. Platelet sequestration in man. I. Methods. J. clin. Invest 43: 843 1964;
    CrossrefPubMedGoogle Scholar
  • 24 Deykin D, Pritzker C. R, Scolnick E. M. Plasma co-factors in adenosine diphosphate-induced aggregation of human platelets. Nature (Lond.) 208: 296 1965;
    PubMedGoogle Scholar
  • 25 Tocantins L. M. In: Blood Coagulation, Hemorrhage and Thrombosis. Tocantins and Kazol Editors. Grune & Stratton; N. Y.: 1964
    Google Scholar
  • 26 Zucker M. B, Peterson J. Inhibition of adenosine diphosphate-induced secondary aggregation and other platelet functions by acetylsalicylic acid ingestion. Proc. Soc. exp. Biol. (N. Y.) 127: 547 1968;
    CrossrefPubMedGoogle Scholar
  • 27 Okonkwo P, Sise H. S. Effect of aspirin in vivo and in vitro on plasma cofactors of platelet aggregation. (To be published.)
    PubMedGoogle Scholar
  • 28 O’Brien J. R. Effect of salicylates on human platelets. Lancet I: 779 1968;
    PubMedGoogle Scholar
  • 29 MacMillan D. G. Effect of salicylates on human platelets. Lancet I: 1151 1968;
    PubMedGoogle Scholar
  • 30 Brinkhous K. M, Read M. S, Mason R. G. Plasma thrombocyte-agglutinating activity and fibrinogen. Synergism with adenosine diphosphate. Lab. Invest 14: 335 1965;
    PubMedGoogle Scholar
  • 31 Born G. V. R, Cross M. J. Effects of inorganic ions and of plasma proteins on the aggregation of blood platelets by adenosine diphosphate. Physiol 170: 397-414 1964;
    CrossrefPubMedGoogle Scholar
  • 32 Gross M. J. Effect of fibrinogen on the aggregation of platelets by adenosine diphosphate. Thrombos. Diathes. haemorrh. (Stuttg.) 12: 524 1964;
    PubMedGoogle Scholar
  • 33 McLean J. R, Maxwell R. E, Hertler D. Fibrinogen and adenosine diphosphate-induced aggregation of platelets. Nature (Lond.) 202: 605 1964;
    CrossrefPubMedGoogle Scholar
  • 34 Caen J. P. Is fibrinogen the plasma co-factor in ADP-induced human platelet aggregation?. Nature (Lond.) 205: 1120 1965;
    CrossrefPubMedGoogle Scholar
  • 35 Gugler E, Lüscher E. F. Platelet function in congenital afibrinogenemia. Thrombos. Diathes. haemorrh. (Stuttg.) 14: 361 1965;
    PubMedGoogle Scholar