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Thromb Haemost 1959; 3(04): 658-674
DOI: 10.1055/s-0038-1654421
Übersichten — Reviews — Revues générales
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Factor VIII Antihemophilic Globulin[*)]

Alfredo Pavlovsky   *)
1   General Review
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Publication History

Publication Date:
12 June 2018 (online)

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Summary

After an historical introduction the author reviews to properties of Factor VIII, its origin and metabolism, methods for its isolation and determination, its rôle in hemostasis and its therapeutic use in cases of hemophilia A and vascular hemophilia A.

*) Synonyms: (In accordance with the International Committee for Standardization of Nomenclature of the Blood Clotting Factors). Factor VIII (Koller)

*) Antihemophilic Globulin ( Patek and Taylor)

*) Antihemophilic Globulin A (Cramer)

*) A. H. F. Antihemophilic Factor (Brinkhous)

*) P. T. F. Plasma Thromboplastic Factor (Ratnoff)

*) P. T. F. A. Plasma Thromboplastic Factor A (Aggeler)

*) T. P. C. Thromboplastic Plasma Component (Shinowara)

*) Facteur Antihemophilique A (Soulier)

*) Thromboplastinogen (Quick)

*) Prothrombokinase (Feissly)

*) Platelet Co-factor (Johnson)

*) Plasmokinin ( Laki )

*) Thrombokatalysin (Lenggenhager).


**) Instituto de Investigaciones Hematologicas de la Academia Nacional de Medicina. Melo 3081. Buenos Aires. Argentina.


 

  • References

  • 1 Addis T. The pathogenesis of hereditary hemophilia. J. Path. Bact 1911; 15: 427
    CrossrefPubMedSearch in Google Scholar
  • 2 Aggeler P. M, White S. G, Glendening M. B, Page E. W, Leake T. B, Bates G. Plasma thromboplastin component (PTC). Proc. Soc. exp. Biol. (N.Y.) 1952; 79: 692
    CrossrefPubMedSearch in Google Scholar
  • 3 Alexander B. The clotting defect in Hemophilia (discussion). The hemophilia and hemophilioid diseases. Ed. Brinkhous; Chapel Hill: 1957: 22
    Search in Google Scholar
  • 4 Alexander B, Landwehr G. Studies of hemophilia II. The Assay of the antihemophilic clot-promoting principle in normal human plasma with some observation on the relative potency of certain plasma fraction. J. clin. Invest 1948; 27: 98
    PubMedSearch in Google Scholar
  • 4a Andino P. A, Pavlovsky A. Tratamiento de la hemofilia. Symposium sobre Factor VIII. Acad. Nac. Med. Bs. As. Agosto. 1959
    PubMedSearch in Google Scholar
  • 5 Bendien W. M, van Creveld S. Onderzoekinger over haemophilie. Maandschr. Kindergeneesk 1936; 5: 179
    PubMedSearch in Google Scholar
  • 6 Bergna L. J, Pavlovsky A. Método para clasificar a los hemofílicos complicados por el desarrollo de un anticoagulante. Rev. Arg. de Hemat. y Hemoterapia 1955; 2: 138
    PubMedSearch in Google Scholar
  • 7 Bergna L. J, Pavlovsky A. Valoración del factor Villi y IX en la hemofilia A y B. Symposium sobre el Factor VIII. Acad. Nac. Med. Bs. As. Agosto. 1959
    PubMedSearch in Google Scholar
  • 7a Bergsagel D. E, Biggs R. The Christmas Factor. Rev. Hémat 1955; 10: 354
    PubMedSearch in Google Scholar
  • 8 Bidwell E. The purification of bovine antihaemophilic Globulin. Brit. J. Haematol 1955; 1: 35
    CrossrefPubMedSearch in Google Scholar
  • 9 Biggs R, Douglas A. S. The thromboplastin generation test. J. clinfl Path 1953; 6: 23
    PubMedSearch in Google Scholar
  • 10 Biggs R, Douglas A. S, Macfarlane R. G, Dacie J. V, Pitney W. R, Merskey C, O’Brien J. R. Christmas disease. Brit. med. Journ 1952; 2: 1378
    CrossrefPubMedSearch in Google Scholar
  • 11 Biggs R, Douglas A. S, Macfarlane R. G. The formation of thromboplastin in human blood. J. Physiol. (Lond.) 1953; 119: 89
    CrossrefPubMedSearch in Google Scholar
  • 12 Biggs R, Eveling S, Richards G. T. The assay of Antihemophilic activity. Brit. J. Haematol 1955; 1: 1
    CrossrefPubMedSearch in Google Scholar
  • 13 Biggs R, Macfarlane R. G. Haemophilia and related conditions. A Survey of 187 cases. Brit. J. Haematol 1958; 4: 1
    CrossrefPubMedSearch in Google Scholar
  • 14 Blombäck M. Studies on antihemophilic globulin. Acta Pediatrica, vol 1958; 47 supl 114
    PubMedSearch in Google Scholar
  • 15 Bounameaux Y. Dosage des facteurs de coagulation contenus dans l’atmosphere plasmatique de plaquettes humaines. Rev. franc. del Et. Clin. et Biol 1957; 2: 52
    PubMedSearch in Google Scholar
  • 16 Brakman P, van Creveld S, Engelsman E, vant Laar I. A, Mochtar I. A, Vender H. A. Deterioration of AHG (Factor VIII) and Fibrinolysis. Thromb. Diath. haem 1959; 3: 1
    PubMedSearch in Google Scholar
  • 17 Brinkhous K. M. A study of the clotting defect in hemophilia: the delayed formation of thrombin. Amer. J. med. Sci 1939; 198: 509
    CrossrefPubMedSearch in Google Scholar
  • 18 Brinkhous K. M. Hemophilia. Bull. N.Y. Acad. Med 1954; 3: 325
    PubMedSearch in Google Scholar
  • 19 Brinkhous K. M. Hemophilia and Hemophilioid Diseases (Int Symp.) Ed. K. M. Brinkhous; Chapel Hill: 1957
    Search in Google Scholar
  • 20 Brinkhous K. M, Penick G. D. Some Systemic alterations in clotting factors following local tissue Injury. Internat. Congr. on Thromb. and Embol. Basel: 1954: 428
    Search in Google Scholar
  • 21 Castex M. R, Paviovsky A. Hemophilic Sang. 1953; 24: 573
    PubMedSearch in Google Scholar
  • 22 Castex M. R, Pavlovsky A, Simonetti C. Contribucion al estudio de la Fisiópatogenia de la hemofilia. Medicina 1944; 5: 16
    PubMedSearch in Google Scholar
  • 23 Cohn E. J, Gurd F. R. N, Surgenor D. M, Barnes B. A, Brown R. K, Deronaux G, Gillespie J. M, Kahurt F. W, Lever W. F, Lin C. H, Mittelman D, Mouton R. F, Schmid R, Uroma E. A system for the separation of the components of human blood: Quantitative procedures for the separation of the protein components of human plasma. J. Amer. chem. Soc 1950; 72: 465
    CrossrefPubMedSearch in Google Scholar
  • 24 Cohn E. J, Strong L. E, Hughes W. L. S, Mulferd D. L, Ashworth J. N, Melir M, Taylor A. L. Preparation and properties of serum and plasma proteins. J. Amer. chem. Soc 1946; 68: 459
    CrossrefPubMedSearch in Google Scholar
  • 25 Cramer R, Flückiger P, Gasser C, Koller F, Loeliger A, Matter M. Hemophilia B. Acta haemat. (Basel) 1953; 10: 65
    CrossrefPubMedSearch in Google Scholar
  • 26 Deutsch E. Treatment of Surgical complications in hemophilia. Hemophilia and Hemophilioid Diseases. Ed. Brinkhous; Chapel Hill: 1957: 204
    Search in Google Scholar
  • 27 Fisch U, Duckert F. Some aspects of Kinetics of the first stages of blood thromboplastin formation. Thromb. Diath. haem 1959; 1: 98
    PubMedSearch in Google Scholar
  • 28 Graham J. B. Biochemical Genetics of blood coagulation. Amer. J. hum. Genet 1956; 8: 63
    PubMedSearch in Google Scholar
  • 29 Graham J. B, Bullock W. B, Barrow E. M. Genetic control of the hemophilioid states. Relat. VII Cong. Soc. Int. Hemat. Rome, Sept. 1958
    Search in Google Scholar
  • 29a Graham J. B, Barrow E. M. The pathogenesis of Hemophilia: An experimental analysis of the anticephalin hypothesis in hemophilic dogs. J. exp. Med 1957; 106: 273
    CrossrefPubMedSearch in Google Scholar
  • 30 Gross J. D, Hartmann R. C, Graham J. B, Bruce Taylor C. Splenectomy in Hemophilia. Bull. Johns Hopk. Hosp 1957; 100: 223
    PubMedSearch in Google Scholar
  • 31 Hecht E. El problema de la hemofilia. Triangulo 1955; 2: 99
    PubMedSearch in Google Scholar
  • 32 Hecht E. Sphingosine discovered as physiological inhibitor of blood clotting. Sixth Annual Symposium on Blood. Wayne State Univers. Detroit. 1957
    PubMedSearch in Google Scholar
  • 33 Hougie C. The rol of factor V in the formation of blood thromboplastin. J. Lab. clin. Med 1957; 50: 61
    PubMedSearch in Google Scholar
  • 34 Jacques L. B, Fisher L. M, Ashwin J. Platelet Serotonin as a factor in hemostasis. Int. Cong. on blood Transfusion. Roma, Sept. 1958
    PubMedSearch in Google Scholar
  • 35 Jorpes E, Nilsson I. M, Blombäck M, Blombäck B. Correction with human plasma Fraction I—0 in Willebrands Disease. VII Congresso della Societá Inter-nazionale di Ematologia. Roma, Sept. 1958
    PubMedSearch in Google Scholar
  • 36 Kekwick R. A, Mackay M. E. The separation of protein fractions from human plasma with ether. Spec. Rep. Serv. Med. Res. Coun. London: 286 1954
    Search in Google Scholar
  • 37 Kekwick R. A, Wolf P. A concentrate of Human antihaemophilic factor its use in six cases of haemophilia. Lancet 1957; 1: 647
    PubMedSearch in Google Scholar
  • 37a Koller F, Krusse G, Lucksinger P. Über eine besondere Form hämor-rhagischer Diathese. Schweiz. med. Wschr 1950; 80: 41
    PubMedSearch in Google Scholar
  • 38 Langdell R. D, Wagner R. H, Brinkhous K. M. Estimation of antihemophilic Activity by the Partial Thromboplastin Time Technic. The Coagulation of Blood, Ed. L. M. Tocantins; N. Y: 1955
    Search in Google Scholar
  • 39 Langdell R. D, Wagner R. H, Brinkhous K. M. Antihemophilic factor (AHF) levels following transfusion of blood plasma and plasma fractions. Proc. Soc. exp. Biol. (N. Y.) 1955; 88: 212
    CrossrefPubMedSearch in Google Scholar
  • 40 Lorand L, Laki K. A simple method for purifying an activator of prothrombin antihemophilic factor. Biochem. Biophys. Acta 1954; 13: 448
    CrossrefPubMedSearch in Google Scholar
  • 41 Lowenburg H, Rubenstone A. J. Hemophilia, experimental data bearing on the effect of glycerinized extracts of visceral hemophilic tissue on the coagulation time of blood. J. Amer. med. Ass 1918; 71: 1196
    CrossrefPubMedSearch in Google Scholar
  • 42 Macfarlane R. G, Biggs R, Bidwell E. Bovine antihemophilic globulin in the treatment of haemophilia. Lancet 1954; 1: 1313
    PubMedSearch in Google Scholar
  • 43 Manteuffel Z. Bemerkungen zur Blutstillung bei Hämophilie. Dtsch. med. Wschr 1893; 19: 665
    Thieme ConnectPubMedSearch in Google Scholar
  • 44 Margolis J. Plasma pain-producing substance and blood clotting. Nature (Lond.) 1957; b 180: 1464
    CrossrefPubMedSearch in Google Scholar
  • 45 Martinez Canaveri A, Pavlovsky A. Participación del Factor VIII en el proceso de la coagulatión. Symposium sobre el Factor VIII. Acad. Nac. Med. Bs. As. Agosto. 1959
    PubMedSearch in Google Scholar
  • 46 Minot G. R, Davidson C. S, Lewis J. H, Tagnon H. J, Taylor F. H. L. The coagulation defect in Hemophilia: The effect in hemophilia of the parenteral administration of a fraction of the plasma globulins rich in fibrinogen. J. clin. Invest 1945; 24: 705
    PubMedSearch in Google Scholar
  • 47 Nilsson I. M, Blombäck M, Jorpes E, Blombäck B, Johansson S. v. Willebrand’s Disease and its correction with Human plasma Fraction 1–0. Acta med. scand. vol. CLIX, Fasc. 1957: III
    Search in Google Scholar
  • 48 Owren P. A. The present state of the converting and accelerating factors in Prothrombin conversion. Internat. Conf. on Thromb. and Embol. Basel: 1954: 65
    Search in Google Scholar
  • 49 Patek A. J, Stetson R. P. The abnormal coagulation of the blood and its relation to the blood platelets. J. clin. Invest 1936; 15: 5
    PubMedSearch in Google Scholar
  • 50 Patek A. J, Taylor F. H. L. Some properties of a substance obtained from human plasma effective in accelerating the coagulation of hemophilic blood. J. clin. Invest 1937; 16: 113
    CrossrefPubMedSearch in Google Scholar
  • 51 Pavlovsky A. Contribution to the Pathogenesis of Hemophilia. Blood 1947; 2: 185
    PubMedSearch in Google Scholar
  • 52 Pavlovsky A. Present concepts of Hemophilia. Blood 1954; 9: 291
    PubMedSearch in Google Scholar
  • 53 Pavlovsky A. Hemophilia. Pren. méd. argent 1958; 65: 2365
    PubMedSearch in Google Scholar
  • 54 Pavlovsky A. Symposium on Hemophilia. VII Cong. Internat. Haemat. Roma, Sept. 1958
    PubMedSearch in Google Scholar
  • 55 Pavlovsky A, Bergna L, Martinez Canaveri A. Thromboplastin in Hemophilia. Sang 1955; 26: 162
    PubMedSearch in Google Scholar
  • 56 Pavlovsky A, Martinez Canaveri A, Andino Pavlovsky A. Emoterapia nella hemofilia. Relato al VII congreso de la Sociedad Internacional de Transfusión de sangre. Roma, Sept. 1958
    PubMedSearch in Google Scholar
  • 57 Pavlovsky A, Martinez Canaveri A, Simonetti C, Arini E. Hemophilia. Comparative study of varieties of A and B. Report VI Congr. Int. Soc. Hem. Boston: 1956
    Search in Google Scholar
  • 58 Pavlovsky A, Mittelman D. G. de, Castellanos H. Interacción de plasma de distintos hemofilicos. Reunión Anual Ins. de Inv. Fis. aplic. a la Pat. Hum. Acad. Nac. Med. Bs. As. Dec. 1950
    PubMedSearch in Google Scholar
  • 59 Pavlovsky A, Pavlovsky M. C. de. Female Haemophilia. Symposium on Hemophilia. VII congreso de la Soc. Int. de Hem. Roma, Sept. 1958
    PubMedSearch in Google Scholar
  • 60 Pavlovsky A, Simonetti C. Determinación del poder coagulante de la fracción globulina extraída de plasmas hemofilicos. An. del Inst. Inv. Acad. Nac. Med 1944; 6: 341
    PubMedSearch in Google Scholar
  • 61 Pavlovsky A, Simonetti C, Andino A. Hemofilia. Considerazioni sulla sua coagulazione. Relato VII Cong. de la Soc. Int. de Hemat. Roma: 1958
    Search in Google Scholar
  • 62 Penick G. D, Brinkhous K. M. Relative stability of plasma antihemophilic factor (AHF) under different conditions of storage. Am. J. med. Sci 1956; 232: 434
    CrossrefPubMedSearch in Google Scholar
  • 63 Pitney W. R. The assay of antikaemophilic globulin (AHG) in plasma. Brit. J. Haematol 1956; II: 3
    PubMedSearch in Google Scholar
  • 64 Pool G. J, Robinson J. Assay of plasma antihemophilic globulin (AHG). Brit. J. Haematol 1959; 5: 17
    CrossrefPubMedSearch in Google Scholar
  • 65 Pool J. G, Spaet T. H. Ethionine induced depresion of plasma antihemophilic globulin in the rat. Proc. Soc. exp. Biol. (N. Y.) 1954; 87: 54
    CrossrefPubMedSearch in Google Scholar
  • 66 Quick A. J. The Hemorrhagic Diseases and the Physiology of Hemostasis. Charles C. Thomas; Springfield, Illinois: 1942
    Search in Google Scholar
  • 67 Quick A. J. Hemorrhagic Diseases. Lea and Febriger; Philadelphia: 1957
    Search in Google Scholar
  • 68 Quick A. J, Daniels E. R, Hussey C. V. Acquired antithromboplastino-genemia (Hemophilia-like disease) with special reference to its diagnosis. J. Lab. clin. Med 1954; 44: 94
    PubMedSearch in Google Scholar
  • 69 Quick A. J, Stanley-Brown M, Bancroft F. W. A study of the coagulation defect in hemophilia and in jaundice. Amer. J. med. Sci 1935; 190: 501
    CrossrefPubMedSearch in Google Scholar
  • 70 Ratnoff O. D, Margolius A. International Symposium on Hemophilia and Hemophilioid Diseases. Ed. Brinkhous; Chapel Hill: 1957: p. 125
    Search in Google Scholar
  • 71 Schulman I. Management of Hemorrhage in hemophilia. Discussion. Hemophilia and Hemophilioid Diseases. Ed. K. M. Brinkhous; Chapel Hill: 1957: 215
    Search in Google Scholar
  • 72 Schulman I, Smith C. H. Hemorrhagic disease in an infant due to deficiency of a previously undescribed clotting factor. Blood 1952; 7: 794
    PubMedSearch in Google Scholar
  • 73 Seegers W. H. Nature of the blood coagulation mechanisms. I. Inter. Conf. on Thromb. and Embol. 1954. Basel: 31
    Search in Google Scholar
  • 74 Seegers W. H, Landaburu R. H, Fenichel L. Isolation of platelet cofactor I from plasma and some properties of the preparation. Amer. J. Physiol 1957; 190: 1
    PubMedSearch in Google Scholar
  • 75 Shinowara G. Y. Studies on the purification of Thromboplastic Plasma component (antihemophilic substance) in human blood. Amer. J. med. Sci 1957; 233: 5
    PubMedSearch in Google Scholar
  • 76 Simonetti C, Casillas G, Pavlovsky A. Valoración de los metodos para purificar el factor VIII. Symposium sobre factor VIII. Acad. Nac. Med. Bs. As Agosto. 1959
    PubMedSearch in Google Scholar
  • 77 Sjölin K. E. Classical Haemophilia (AHF deficiency) and Christmas Factor (PTC) deficiency as Simultaneous defects. Acta med. scand 1957; 159: 7
    PubMedSearch in Google Scholar
  • 78 Sjölin K. E. A group of AHF deficient patients, corrected by adsorbed serum. Trans of the 6th. Congr. of the Europ. Soc. of Haemat. 1957: 164
    Search in Google Scholar
  • 79 Soulier J. P. Un nouvel adsorbant des facteurs de coagulation. La Bentonite. Rev. Hémat 1959; 14: 26
    PubMedSearch in Google Scholar
  • 79a Soulier J. P. Standardization of So-called Antihemophilic Fractions. Hemophilia and Hemophilioid diseases. Ed. Brinkhous; Chapel Hill: 1957: 18
    Search in Google Scholar
  • 80 Soulier J. P, Gobbi F, et Larrieu M. J. Separation du Fibrinogene et du facteur antihemophilique A. Rev. Hématol 1957; 12: 481
    PubMedSearch in Google Scholar
  • 81 Soulier J. P, Larrieu M. J. Nouvelle méthode de diagnostic de l’hemophilie: dosage des facteurs antihemophiliques A et B. Sang 1953; 24: 205
    PubMedSearch in Google Scholar
  • 82 Soulier J. P, Wartelle O, Menachee D. Hageman Trait and PTA deficiency; the rol of contact of blood with glass. Brit. J. Haematol 1959; 5: 121
    CrossrefPubMedSearch in Google Scholar
  • 83 Spaet T. H, Aggeler P. M, Richards M. O. Prophylactic management of Hemophilia and PTC deficiency. Hemophilia and Hemophilioid diseases. Ed. Brink-hous; Chapel Hill: 1957: 11
    Search in Google Scholar
  • 84 Spaet T. H, Kinsell B. G. Properties of bovine antihemophilic factor. Proc. Soc. exp. Biol. (N. Y.) 1953; 84: 314
    CrossrefPubMedSearch in Google Scholar
  • 85 Taylor F. H. L, Davidson C. S, Tagnon H. J, Adams M. A, Mac-Donald A. H, Minot G. R. Studies in blood coagulation: the coagulation properties of certain globulin fractions of normal human plasma “in vitro”. J. clin. Invest 1945; 24: 698
    CrossrefPubMedSearch in Google Scholar
  • 85a Tocantins L. M, Caroll R. T, Holburn R. H. The clot accelerating effect of dilution on blood and plasma; relation to the mechanism of coagulation of normal and hemophilic blood. Blood 1951; 6: 720
    PubMedSearch in Google Scholar
  • 86 Verstraete M. De Vorming van Plasma Thromboplastine — Arscia Uitgaven Stoofsträat 60. Brussel: 1955
    Search in Google Scholar
  • 87 Verstraete M, Vandenbroucke J. Combined antihemophilic Globulin and Christmas factor deficiency in haemophilia. Brit. Med. J 1955; 11: 1533
    PubMedSearch in Google Scholar
  • 88 Wagner R. H, Thelin Murray G. Antihemophilic Factor: Current Status of Purification Chemical and Physical characterization. Hemophilia and Hemophilioid Diseases. Ed; Brinkhous Chapel Hill: 1957: 3
    Search in Google Scholar
  • 89 Winterstein A, Marbet R, Strassle R. Isolation, Stability and assay of Antihemophilic globulin. Hemophilia and Hemophilioid Diseases. Ed; Brinkhous, Chapel Hill: 1957: 11
    Search in Google Scholar
  • 90 Wolf P. Studies of Temperature and pH stability of Human antihaemophilic Factor (AHF) in plasma and in a concentrate. Brit. J. Haematol 1959; 5: 169
    CrossrefPubMedSearch in Google Scholar