Thromb Haemost 1971; 25(01): 147-156
DOI: 10.1055/s-0038-1654289
Originalarbeiten – Original Articles – Travaux Originaux
Schattauer GmbH

Prothrombin Level and Activity in the Abnormal Factor X (Factor X Friuli) Hemorrhagic Disorder

A Girolami
1   University of Padua Medical School, Institute of Medical Semeiotics, Padua, Italy
,
A Sticchi
1   University of Padua Medical School, Institute of Medical Semeiotics, Padua, Italy
,
A Brunetti
1   University of Padua Medical School, Institute of Medical Semeiotics, Padua, Italy
› Author Affiliations
Further Information

Publication History

Publication Date:
24 July 2018 (online)

Summary

Prothrombin has been assayed by means of 7 different techniques in the patients so far known to have the abnormal factor X (factor X Friuli) coagulation disorder. The methods were: classical one-stage method, Iowa two-stage method, Oxford two-stage method, a modified Hjort’s method using Stypven-Cephalin, a modified Jobin and Esnouf’s method using Tiger venom, the Staphilocoagulase method and an immunological method with anti-human prothrombin rabbit serum. In all cases a normal prothrombin level was found. In the two-stage procedures a normal thrombin activity was obtained only when normal serum was added to the system.

A good correlation was found between the several methods used.

In subjects known to be heterozygote for the abnormal factor X coagulation disorder, prothrombin level and activity has been found to be normal too. These data suggest that the abnormal factor X (factor X Friuli) coagulation disorder is due to a distinct factor X protein anomaly unaccompanied by detectable changes in the prothrombin level or activity.

 
  • References

  • 1 Aggeler P. M, White S. G, Glendening M. B, Page E. W, Leake T. B, Bates G. Plasma Thromboplastin Component (PTC) deficiency. A new disease resembling hemophilia. Proc. Soc. exp. Biol. (N. Y.) 79: 692 1952;
  • 2 Alexander B, Goldstein B, Landwehr G, Cook G. D. Congenital SPCA deficiency: a hither to unrecognised coagulation defect with hemorrhage rectified by serum and serum fractions. J. clin. Invest 30: 596 1951;
  • 3 Bachman F, Duckert F, Koller F. The Stuart-Prower factor assay and its clinical significance. Thrombos. Diathes. haemorrh. (Stuttg) 02: 24 1958;
  • 4 Biggs R, Douglas A. S, Mac Farlane B. G, Dacie J. V, Pitney W. B, O’Brien J. B. Christmas disease, a condition previously mistaken for hemophilia. Brit. J. Med I: 1378 1952;
  • 5 Biggs B, Douglas A. S. The measurement of prothrombin in plasma. A case of prothrombin deficiency. J. clin. Path 06: 15 1953;
  • 6 Biggs B, Mac Farlane B. G. Human Blood Coagulation and its disorders. Davis and Co; 3rd Edition. Philadelphia: 1962
  • 7 Borchgrevink G. F, Egeberg O, Pool J. P, Skulason T, Stormorken H, Waaler B. A study of a case of congenital hypoprothrombinemia. Brit. J. Haemat 05: 294 1959;
  • 8 Cartwright G. E. Diagnostic Laboratory Hematology. Grune and Stratton; 3rd Edition. New York: 1963
  • 9 Denson K. W. The specific assay of Prower-Stuart factor and factor VII. Acta haemat. (Basel) 25: 105 1961;
  • 10 Denson K. V. The use of antibodies in the study of blood coagulation. Blackwell Scient. Pub; Oxford: 1967
  • 11 Girolami A, Lazzarin M, Scarpa B, Brunetti A. A study of another case of congenital hypothrombinemia. Acta haemat. (Basel) 44: 164 1970;
  • 12 Girolami A, Molaro G, Lazzarin M, Scarpa B, Brunetti A. A new congenital hemorrhagic condition due to the presence of an abnormal factor X (factor X Friuli). A study of a large Kindred. Brit. J. Haemat 19: 179 1970;
  • 13 Girolami A, Molaro G, Lazzarin M. D, Scarpa B, Brunetti A. Congenital hemorrhagic condition similar but not identical to factor X deficiency. Scand. J. Haemat 07: 91 1970;
  • 14 Girolami A, Molaro G, Lazzarin M, Scarpa B. Una nuova coagulopatia emorragica congenita probablimente dovuta alla presenza di un fattore X abnorme. Studio preliminare. Min. Med 60: 4939 1969;
  • 15 Hjort P, Bapaport S. I, Owren P. A. A simple specific one-stage prothrombin assay using Russell’s viper venom in cephalin suspension. J. Lab. clin. Med 46: 89 1955;
  • 16 Hougie C, Barrow E. M, Graham J. B. Stuart clotting defect. I. Segregation of an hereditary hemorrhagic state from the heterogeneus group heretofore called “stable factor” (SPCA, proconvertin, factor VII) deficiency. J. clin. Invest 36: 485 1957;
  • 17 Jobin F, Esnouf M. P. Coagulant activity of Tiger snake (notechis scutatus scutatns) venom. Nature 211: 873 1966;
  • 18 Josso F, Prou-Wartelle G, Soulier J. P. Etude d’un cas d’hypoprothrombinémie congénitale. Nouv. Rev. franç. Hémat 02: 647 1962;
  • 19 Kattlove M. E, Shapiro S. S, Svivack M. Hereditary Prothrombin deficiency. New Engl. J. Med 282: 57 1970;
  • 20 Lewis J. H, Ferguson J. H, Fresh J. W, Zucker M. B. Primary hemorrhagic diseases. J. Lab. clin. Med 49: 211 1957;
  • 21 Malhotra O. P, Garter J. R. Prothrombin in factor X deficiency. J. Lab. clin. Med 75: 120 1970;
  • 22 Mancini S, Vaerman J. P, Carbonara O, Heremans J. F. A simple radial diffusion method for the immunological quantitation of proteins. In: Peters H. (Ed.) Proc. Xlth Colloquium Protides of the Biolog. Fluids, Brugge; 1963: 370 Elzevier, Amsterdam: 1964
  • 23 Miale J. B. Laboratory Medicine. Hematology. C. V. Mosby Co; St. Louis: 1967
  • 24 Quick A. J. The Physiology and pathology of hemostasis. Lea and Febiger; Philadelphia: 1955
  • 25 Quick A. J, Hussey G. V. Hereditary Hypoprothrombinemias. Lancet I: 173 1962;
  • 26 Seegers W. H. Prothrombin. Harward University Press; Cambridge (Mass): 1962
  • 27 Seegers H. W. An outline of basic enzymology in blood clotting. Minn. Med 49: 171 1966;
  • 28 Seegers W. H, Murano G, Mc Coy L. Structural changes in prothrombin during activation, a theory. Thrombos. Diathes. haemorrh. (Stuttg) 23: 26 1970;
  • 29 Shapiro S. S, Martinez J, Holburn R. H. Congenital dysprothrombinemia: an inherited structural disorder of human prothrombin. J. clin. Invest 48: 2251 1969;
  • 30 Scheidegger J. J. Une micro-méthode de l’immuno-électrophorese. Int. Arch. Allergy 07: 103 1955;
  • 31 Soulier J. P, Prou-Wartelle O. Etude comparative des taux de cofacteur de la staphylo- coagulase (C.R.F.) et des taux de facteur II (prothrombine) dans diverses conditions. Nouv. Rev. franç. Hémat 06: 623 1966;
  • 32 Soulier J. P. La thrombine-coagulase. Ann. Biol. Clin 27: 285 1969;
  • 33 Telfer T. P, Denson K. W, Wright D. R. A new Coagulation defect. Brit. J. Haemat 11: 308 1956;
  • 34 Ware A. G, Seegers W. H. Two-stage procedure for the quantitative determination of prothrombin concentration. Amer. J. clin. Path 19: 471 1949;