Differentiation of the Factor VII Complex

Summary

A factor recently described and for which the designation Stuart-Prower Factor is proposed, was investigated. It is detected by the usual Factor VII assay but differs from this factor (Matching experiments with plasma and serum of Owren’s Factor VII deficient patient). Stuart-Prower factor is necessary for the conversion prothrombin-thrombin with tissue thromboplastin as well as for the formation of blood thromboplastin. Therefore lack of Stuart-Prower factor produces abnormal Quick’s prothrombin time (not normalized with Russell’s viper venom [Stypven]), and also abnormal prothrombin consumption, abnormal recalcification time and abnormal thromboplastin generation with serum. Matching experiments with plasma and serum of the patients of Graham and Hougie, Telfer Denson and Wright, and Beaumont and Bernard show that we are dealing with the same defect. Physiological and physico-chemical properties are indicated. The possible relationship between Stuart-Prower factor and Factor X is discussed. A critical review with 59 cases of the so-called Factor VII deficiency is given. Investigation of the family of our patient Delia B. indicates that the mode of inheritance is an intermediate autosomal one.

 

• References

• 1 Ackroyd J. F. The Function of F. VII. Brit. J. Haemat. 2: 397 1956;
• 2 Alexander B, Goldstein R, Landwehr G, and Cook C. D. Congenital SPCA-Deficiency: A hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fractions. J. clin. Invest. 30: 596 1951;
• 3 Alexander B. Discussion on Owren (46), p. 112 1952
• 4 Bachmann F, Duckert F, Flückiger P, Hitzig W, and Koller F. Über einen neuartigen kongenitalen Gerinnungsdefekt (Mangel an Stuartfaktor). Thromb. Diath. haem. 1: 87 1957;
• 5 Barnett C. P. Hemorrhagic Diathesis due to Factor VII deficiency. A. M. A. Arch, intern. Med. 99: 280 1957;
• 6 Beaumont J. L, et Bernard J. Syndrome hémorrhagique congénital dû au défaut du facteur de coagulation récemment isolé sous le nom de Facteur VII, Convertine, S. P. CA. Acta med. scand. 145: 200 1953;
• 7 Bell W. N, and Alton H. G. A brain extract as a substitute for platelet suspensions in the thromboplastin generation test. Nature (Lond.) 174: 880 1954;
• 8 Bell W. N, and Alton H. G. Christmas disease associated with Factor VII deficiency. Case report with family survey. Brit. med. J. 1: 330 1955;
• 9 van Belle C. J. Parahaemophilie. Academisch Proefschrift. Utrecht 1952
• 10 Biggs R. Some observations on the blood of patients with ‘Factor VIP deficiency. Brit. J. Haemat. 2: 412 1956;
• 10a Brinkhous K. M, Langdell R. D, Penick G. D, Graham J. B, and Wagner R. H. Newer approaches to the study of hemophilia and hemophiloid states. J. Amer. med. Ass. 154: 481 1954;
• 11 Chevallier P, Bernard J, Fiehrer A, Bilski-Pasquier G, Samama M, et Cerf M. Deux cas d’hypoconvertinémie familiale. Sang 26: 650 1955;
• 12 Choremis C, Padiatellis C, Tsevrenis I, Hadjidimitriou E, Priovolos I, et Mandalaki T. A propos d’un cas d’hypoproconvertinémie congénitale. Helv. paediat. Acta 11: 301 1956;
• 13 Clauss A. Gerinnungsphysiologische Schnellmethode zur Bestimmung des Fibrinogens. Acta haemat. (Basel) 17: 237 1957;
• 14 van Crefeld S, Veder H, and Blans M. Congenital hypoproconvertinemia. Ann. paediat. (Basel) 187: 373 1956;
• 15 Crockett Jr. C. L, Shotton D, Craddock Jr. C. G, and Leavell S. S. Hypoprothrombinemia: studies of a case of the idiopathic type and the effect of serum administration. Blood 4: 1298 1949;
• 16 Duckert F, Flückiger P, Isenschmid H, Matter M, Vogel-Meng J, and Koller F. A modification ot the thromboplastin generation test. Acta haemat. (Basel) 12: 197 1954;
• 17 Duckert F, Flückiger P, and Koller F. Le rôle du facteur X dans la formation de la thromboplastine sanguine. Rev. Hémat. 9: 489 1954;
• 18 Duckert F, Flückiger P, Matter M, and Koller F. Clotting Factor X. Physiologic and physicochemical properties. Proc. Soc. exp. Biol. (N. Y.) 90: 17 1955;
• 19 Frick P. G, and Hägen P. S. Congenital familial deficiency of the stable prothrombin conversion factor. Restudy of a case originally reported as ‘idiopathic hypoprothrombinemia’. J. Lab. clin. Med. 42: 212 1953;
• 20 Geiger M, Duckert F, and Koller F. Quantitative Bestimmungen von Faktor VIII und IX bei Blutersippen. 5the Congr. Europ. Soc. Haemat. Freiburg. p. 413 1956
• 21 Gillot F, Venezia R, Boineau N, et Colonna M.-O. Un cas d’hypoconvertinémie congénitale chez le nourrisson. Pédiatrie 11: 439 1956;
• 22 Giordano A. S. Idiopathic hypoprothrombinemia. Amer. J. clin. Path. 13: 285 1943;
• 23 Graham J. B, and Hougie C. The Stuartfactor (hypoproconvertinemia?). The clotting defect and mode of inheritance. 6the Congr. Int. Soc. Haemat. Boston, p. 327 1956
• 24 Graham J. B, Barrow E, and Hougie C. Stuart clotting defect. II. Genetic aspects of a ‘new’ hemorrhagic state. J. clin. Invest. 36: 497 1957;
• 25 Greig H. B. W, Falcke H. C, Simon M, and Cohen H. Congenital Factor VII deficiency. Arch. Dis. Childh. 31: 293 1956;
• 26 Greig H. B. W, and Tattersall J. C. Evidence for the existence of a third serum clotting factor. Brit. J. Haemat. 2: 421 1956;
• 27 Hagen P. S, and Watson C. J. Idiopathic (familial) hypoprothrombinemia. J. Lab. clin. Med. 33: 542 1948;
• 28 Hicks N. D. A coagulation disorder due to a Factor VH-like defect. Med. J. Austr. 2: 331 1955;
• 29 Hjort P, Rapaport S. I, and 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;
• 30 Hougie C, and Graham J. B. The blood clotting role and mode of inheritance of the Stuart factor. 6the Congr. Int. Soc. Blood Transf. Boston, p. 80 1956
• 31 Hougie C. The role of Russell’s viper venom on the Stuart clotting defect. Proc. Soc. exp. Biol. 93: 570 1956;
• 32 Hougie C, Barrow E, and Graham J. B. Stuart clotting defect. I. Segregation of an hereditary hemorrhagic state from the heterogeneous group heretofore called ‘Stable Factor’ (SPCA, proconvertin, Factor VII) deficiency. J. clin. Invest. 36: 485 1957;
• 33 Hule V, Sabacky J, and Saxl O. Ein Fall von angeborenem Mangel von Faktor VII (SPCA-Mangel, Hypoproconvertinemia congenita). Helv. paediat. Acta 10: 419 1955;
• 34 Jenkins J. S. Haemorrhagic diathesis due to a deficiency of Factor VII. J. clin. Path. 7: 29 1954;
• 35 Jenkins J. S. The thromboplastic activity of Russell’s viper venom and its relationship to Factor VII. J. clin. Path. 7: 287 1954;
• 36 Jürgens J. Congenital Factor VII deficiency — a haemophilia-like haemorrhagic disease — and its response to a plasma fraction (Behring’s accelerator 76). Abstract in 4th Congr. Europ. Soc. Haemat. Amsterdam, p. 54 1953
• 37 Jürgens J. Kongenitaler Faktor VII (SPCA)-Mangel als Ursache einer hämophilie-artigen hämorrhagischen Diathese. Acta haemat. (Basel) 16: 181 1956;
• 38 Koch Fr, Schultze H. E, Schwick G, and Beller F. K. Beobachtungen bei angeborenem Faktor VIl-Mangel (Hypokonvertinämie). Zschr. f. Kinderh. 76: 208 1955;
• 39 Koller F, Loeliger A, and Duckert F. Experiments on a new clotting factor (Factor VII). Acta Haemat. 6: 1 1951;
• 39a Koller F. Die Physiologie der Blutgerinnung. Arch, exper. Pathol, and Pharmak. 222: 90 1954;
• 40 Koller F. Is hemophilia a nosologic entity?. Blood 9: 286 1954;
• 40a Koller F. Le facteur X. Rev. Hémat. 10: 362 1955;
• 41 Landwehr G, Lang H, and Alexander B. Congenital hypoprothrombinemia. A case study with particular reference to the role of non-prothrombin factors in the conversion of prothrombin. Amer. J. Med. 8: 255 1950;
• 42 Lewis J. H, Fresh J. W, and Ferguson J. H. Congenital hypoproconvertinemia. Proc. Soc. exp. Biol. (N. Y.) 84: 651 1953;
• 43 Long L. A, Letendre P, and Colpron G. Hypoproconvertinémie congénitale. Acta Haemat. 13: 242 1955;
• 44 Marciniakówna E, Krakowska J, Bober S, and Safarzynska L. Przypadek skazy krwotocznej wskutek niedoboru akcleratora konwersji protrombiny. Pol. Tyg. lek. 8: 1601 1953;
• 45 Newcomb T. The role of known coagulation factors in the thromboplastin generation test. J. clin. Invest, in press
• 46 Owren P. A. New clotting factors. Trans. 5 th Conf. Blood Clotting and Allied Problems, p. 92. Josiah Macy, Jr. Foundation. New York 1952
• 47 Owren P. A. Prothrombin and accessory factors: clinical significance. Amer. J. Med. 14: 201 1953;
• 47a Owren P. A, Newcomb T, and Hjort P. Personal communications to R. Biggs 10
• 48 Pitney W. R, and Dacie J. V. A simple method of studying the generation of thrombin in recalcified plasma. Application in the investigation of haemophilia. J. clin. Path. 6: 9 1953;
• 49 Quick A. J. The prothrombin in hemophilia and in obstructive jaundice. J. biol. Chem. 109: 523 1935;
• 50 Quick A. J, Pisciotta A. V, and Hussey C. V. Congenital hypoprothrom-binemic states. Arch. int. Med. 95: 2 1955;
• 51 Rapaport S. I, Aas K, and Owren P. A. The clotting action of Russell’s viper venom. Blood 9: 1185 1954;
• 52 Serafini U. M, and Pericoli F. Sindrome emorragica di tipo emofilio da difetto congenito di Fattore VII. Boll. Soc. Ital. Emat. 4: 70 1956;
• 53 Soulier J. P, Alagille D, Martin C, et Buhot S. Un cas d’hypocon-vertinémie congénitale. Sang 26: 660 1955;
• 54 Stefanovic S, Milosavljevic A, and Stefanovic R. Deux cas d’hypo-convertinémie congénitale. Sang 26: 315 1955;
• 55 Stein H. B, and Abrahams O. L. Combined mild PTC (Plasma thromboplastin component) and Factor VII deficiencies. S. Afr. J. med. Sci. 21: 13 1956;
• 56 Telfer T. P, Denson K. W, and Wright D. R. A ‘new’ coagulation defect. Brit. J. Haemat. 2: 308 1956;
• 57 de Vries S. L, Kellenborg H. K, and van der Pool E. T. Haemorrhagische diathese door tekort aan Factor VII (hypoproconvertinaemia). Ned. T. Geneesk. 98: 2987 1954;
• 58 de Vries S. I, Kettenborg H. K, and van der Pool E. T. Haemorrhagic diathesis due to a deficiency of factor VII. (Hypoproconvertinaemia). Acta haemat. (Basel) 14: 43 1955;
• 59 Wurzel H. A, Roth K, and Zubrow S. Mild familial hypoproconvertinemia J. Lab. clin. Med. 44: 403 1954;