Download PDF
Thromb Haemost 1971; 25(03): 438-446
DOI: 10.1055/s-0038-1654318
Originalarbeiten – Original Articles – Travaux Originaux
Schattauer GmbH

Major Surgery in a Subject with Factor V Deficiency

Cholecystectomy in a Parahaemophilic Woman and Review of the Literature
E. J Melliger
1   Blood Coagulation and Fibrinolysis Laboratory (Head: PD Dr. F. Duckert) of the University Department of Medicine (Head: Prof. Dr. F. Koller), Bürgerspital, Basel ( Switzerland)
,
F Duckert
1   Blood Coagulation and Fibrinolysis Laboratory (Head: PD Dr. F. Duckert) of the University Department of Medicine (Head: Prof. Dr. F. Koller), Bürgerspital, Basel ( Switzerland)
› Author Affiliations
Further Information

Publication History

Publication Date:
28 June 2018 (online)

Also available at
    Permissions and Reprints

Summary

A further case of parahaemophilia is reported. One year after the correct diagnosis had been made the patient had to undergo cholecystectomy which was performed under prophylactic substitutive treatment with fresh plasma at a factor V level of 31 %. A minimal factor V level of 11 to 12% was maintained throughout the first week after operation. There was no abnormal postoperative bleeding. The half disappearance time of factor V was found to be about 12 h. Infusion of equivalent amounts of fresh plasma supplied a higher yield of factor V in the patient’s plasma before operation than postoperatively what may be explained by an increased diffusion of factor V into the intercellular space resulting from a postoperatively increased capillar permeability. The results are compared with those of other authors.

 

  • References

  • 1 Alexander B, Goldstein R. Parahemophilia in three siblings (Owren’s disease). With studies on certain plasma components affecting prothrombin conversion. Amer. J. Med 13: 255 1952;
    CrossrefPubMedSearch in Google Scholar
  • 2 Barbieri U, Gobbi F, Ascari E. Deficit congenito associato di globulina antiemofilica (fattore VIII) e di proaccelerina (fattore V) in due fratelli. Minerva Med. (Torino) 56: 4464 1965;
    PubMedSearch in Google Scholar
  • 3 Borchgrevink C. F, Owren P. A. The hemostatic effect of normal platelets in hemophilia and factor Y deficiency. The importance of clotting factors adsorbed on platelets for normal hemostasis. Acta med. scand 170: 375 1961;
    PubMedSearch in Google Scholar
  • 4 Borchgrevink G. F, Owren P. A. Surgery in a patient with factor V (proaccelerin) deficiency. Acta med. scand 170: 743 1961;
    PubMedSearch in Google Scholar
  • 5 Bowie E. J. W, Thompson Jr. J. H, Didisheim P, Owen Jr. G. A. Disappearance rates of coagulation factors : transfusion studies in factor-deficient patients. Transfusion 07: 174 1967;
    CrossrefPubMedSearch in Google Scholar
  • 6 Brennan M. J, Monto R. W, Shafer H. G. Hemorrhagic diathesis due to Ac-globulin deficiency. Amer. J. clin. Path 22: 150 1952;
    PubMedSearch in Google Scholar
  • 7 Brink A. J, Kingsley G. S. A familial disorder of blood coagulation due to deficiency of the labile factor. Quart. J. Med. N.S 21: 19 1952;
    PubMedSearch in Google Scholar
  • 8 Brinkhous K. M, Langdell R. D, Penick G. D, Graham J. B, Wagner R. H. Newer approaches to the study of hemophilia and hemophiloid states. JAMA 154: 481 1954;
    CrossrefPubMedSearch in Google Scholar
  • 9 Brown Jr. I. W, Smith W. W, Howse R. M. Surgical management in hemophilia and the hemophiloid diseases. Ann. Surg 149: 721 1959;
    CrossrefPubMedSearch in Google Scholar
  • 10 Ghrobâk L, Bartos V, Brzek V, Hnizdovâ D. Coagulation properties of human thoracic duct lymph. Amer. J. med. Sci 253: 69 1967;
    CrossrefPubMedSearch in Google Scholar
  • 11 Goelho E, Verrastro T, Jamra M. Parahemofilia. A presentaçao de caso de deficiença de factor V. Rev. Hosp. Clin 12: 216 1957;
    PubMedSearch in Google Scholar
  • 12 Deutsch E, Fleischhacker H. Zur Differentialdiagnose der Parahämophilie. Z. klin. Med 149: 493 1952;
    PubMedSearch in Google Scholar
  • 13 De Vries A, Matoth Y, Shamir Z. Familial congenital labile factor deficiency with syndactylism. Investigations on the mode of action of the labile factor. Acta haemat. (Basel) 05: 129 1951;
    PubMedSearch in Google Scholar
  • 13a Duckert F. Le diagnostic des coagulopathies. Röntgen- und Laborpraxis 13: 156 171, 203, 224 1960;
    PubMedSearch in Google Scholar
  • 14 Fantl P. Parahemophilia (proaccelerin deficiency). Occurence and biochemistry. In: Brinkhous K. M. (Ed.), Hemophilia and hemophiloid diseases. 79 University of North Carolina Press; Chapel Hill: 1957
    Search in Google Scholar
  • 15 Field J. B, Ware A. G. Studies on parahemophilia. J. clin. Invest 33: 932 1954;
    PubMedSearch in Google Scholar
  • 16 Frank E, Bilhan N, Ekren H. Die Parahämophilie (Owren), eine neue Form der hämorrhagischen Diathese. Acta haemat. (Basel) 03: 70 1950;
    CrossrefPubMedSearch in Google Scholar
  • 17 Friedman I. A, Quick A. J, Higgins F, Hussey G. V, Hickey M. H. Hereditary labile factor (factor Y) deficiency. JAMA 175: 370 1961;
    CrossrefPubMedSearch in Google Scholar
  • 18 Gobbi F, Ascari E, Barbieri U. Deficit congenito di proaccelerina (paraemofilia di Owren). Minerva Med. (Torino) 51: 78 1960;
    PubMedSearch in Google Scholar
  • 19 Heni F, Kraus I. Angeborener Faktor-Y-Mangel (Owren’sche Krankheit). Klin. Wschr 32: 633 1954;
    CrossrefPubMedSearch in Google Scholar
  • 20 Hjort P, Rapaport S. I, Owren P. A. Evidence that platelet accelerator (platelet factor I) is absorbed plasma proaccelerin. Blood 10: 1139 1955;
    PubMedSearch in Google Scholar
  • 21 Hörder M. H. Isolierter Faktor-V-Mangel, bedingt durch einen spezifischen Hemmkörper. Acta haemat. (Basel) 13: 235 1955;
    CrossrefPubMedSearch in Google Scholar
  • 22 Hörder M. H, Hiemeyer V. Kongenitaler Faktor-V-Mangel. Eine klinische und experimentelle Studie unter Berücksichtigung der bisher veröffentlichten Fälle von Parahämophilie. Med. Klin 55: 2042 1963;
    PubMedSearch in Google Scholar
  • 23 Iversen T, Bastrup-Madsen P. Congenital familial deficiency of factor V (parahemophilia) combined with deficiency of antihemophilic globulin. Brit. J. Haemat 02: 265 1956;
    CrossrefPubMedSearch in Google Scholar
  • 24 Jones J. H, Rizza G. R, Hardisty R. M, Dormandy K. M, McPherson J. G. Combined deficiency of factor V and factor VIII (antihaemophilic globulin) A report of three cases. Brit. J. Haemat 08: 120 1962;
    CrossrefPubMedSearch in Google Scholar
  • 25 Kagami M, Morita H. Parahaemophilia, the report of one family from Japan. Acta haemat. (Basel) 35: 102 1966;
    CrossrefPubMedSearch in Google Scholar
  • 25a Kappeler R. Das Verhalten von Faktor V im Serum unter normalen und pathologischen Bedingungen. Z. klin. Med 153: 103 1955;
    PubMedSearch in Google Scholar
  • 26 Kingsley G. S. Familial factor V deficiency; the pattern of heredity. Quart. J. Med. N.S 23: 323 1954;
    PubMedSearch in Google Scholar
  • 27 Kwa S. B, Chen Y. F. Congenital factor V deficiency — report of a case. Singapore med. J 06: 184 1965;
    PubMedSearch in Google Scholar
  • 28 Larrieu M. J, Caen J, Grenet P, Gayla J, Bernard J. Hypoaccélérinémie congénitale (parahémophilie d’Owren). Sang 27: 117 1956;
    PubMedSearch in Google Scholar
  • 29 Lewis H. J, Ferguson J. H. Hypoproaccelerinemia. Blood 10: 351 1955;
    PubMedSearch in Google Scholar
  • 30 Lopez-Botet E, Vilar E. Dos casos de parahemofilia por carenzia de factor V y VII de Owren. Rev. clin, espan 58: 220 1955;
    PubMedSearch in Google Scholar
  • 31 Lopez V, Pflugshaupt R, Wirthner H, Bütler R. Hereditärer Faktor-V-Mangel (Parahämophilie) in einer Schweizer Familie. Schweiz, med. Wschr 99: 1354 1969;
    PubMedSearch in Google Scholar
  • 32 Marx R. Konstitutionelle Pseudohypoprothrombinämien. Aerztl. Forsch 04: 567 1950;
    PubMedSearch in Google Scholar
  • 33 Miller S. P. Coagulation dynamics in factor V deficiency: a family study with a note on the occurence of thrombophlebitis. Thrombos. Diathes. haemorrh. (Stuttg) 13: 500 1965;
    PubMedSearch in Google Scholar
  • 34 O’Brien J. R. Factor V in blood coagulation in vitro and a report of a case of factor V deficiency. Brit. J. Haemat 04: 210 1958;
    CrossrefPubMedSearch in Google Scholar
  • 35 Oeri J, Matter M, Isenschmid H, Hauser F, Koller F. Angeborener Mangel an Faktor V (Parahämophilie), verbunden mit echter Hämophilie A bei zwei Brüdern. Bibl, paediatr 58: 575 1954;
    PubMedSearch in Google Scholar
  • 36 Outeirino JHernandez, Fayos JSânchez, Paniagua G, Avendano LHernando, Jaca MAguirre. Parahemophilia defecto congenito de factor V. Rev. clin. esp 101: 56 1966;
    PubMedSearch in Google Scholar
  • 37 Owen Jr. C. A, Cooper T. Parahemophilia. Arch, intern. Med 95: 194 1955;
    CrossrefPubMedSearch in Google Scholar
  • 38 Owren P. A. Parahemophilia. Haemorrhagic diathesis due to absence of a previously unknown clotting factor. Lancet I: 446 1947;
    PubMedSearch in Google Scholar
  • 39 Philips L. L, Little W. A. Factor V deficiency in obstetrics. Obst. Gynecol 19: 507 1962;
    PubMedSearch in Google Scholar
  • 40 Redner B, Scalettar H, Weiner M. Parahemophilia (Owren’s disease). Pediatrics 12: 5 1953;
    PubMedSearch in Google Scholar
  • 41 Rush B, Ellis H. The treatment of patients with factor V deficiency. Thrombos. Diathes. haemorrh. (Stuttg) 14: 74 1965;
    PubMedSearch in Google Scholar
  • 42 Sacks M. S, Raccuglia G. Hereditary deficiency of proaccelerin (parahemophilia). A familial study. J. Lab. clin. Med 46: 98 1955;
    PubMedSearch in Google Scholar
  • 43 Sailer S, Hinrichs R. Isolierter kongenitaler Faktor-V-Mangel als Ursache einer hämorrhagischen Diathese. Wien. Z. inn. Med 40: 10 1959;
    PubMedSearch in Google Scholar
  • 44 Saint-Paul B, Chassaigne M. Déficit majeur en proaccélérine (Facteur V) d’origine congénitale « Maladie d’Owren ». Gaz. méd. France 73: 707 1966;
    PubMedSearch in Google Scholar
  • 45 Seibert R. H, Margolius A, Ratnoff O. D. Observations on hemophilia, parahemophilia and coexistent hemophilia and parahemophilia. J. Lab. clin. Med 52: 449 1958;
    PubMedSearch in Google Scholar
  • 46 Soulier J. P. Les indications respectives du sang frais et du sang conservé dans les déficits en facteurs de coagulation. Transfusion 07: 253 1964;
    CrossrefPubMedSearch in Google Scholar
  • 47 Soulier J-P, Prou-Wartelle O, Weilland G, Ménaché D. Déficit congénital en proaccélérine (facteur V). Quelques données nouvelles. Thrombos. Diathes. haemorrh. (Stuttg) 02: 250 1958;
    PubMedSearch in Google Scholar
  • 48 Stohlman Jr. F, Harrington W. J, Moloney W. G. Parahemophilia (Owren’s disease); report of a case in a woman with studies on other members of her family. J. Lab. clin. Med 38: 842 1951;
    PubMedSearch in Google Scholar
  • 49 Tango M, Ujiie A, Ishikawa N, Yasukouchi T. Familial congenital factor V deficiency (parahemophilia). J. Jap. Soc. intern. Med 54: 816 1965;
    CrossrefPubMedSearch in Google Scholar
  • 50 Webster W. P, Roberts H. R, Penick G. D. Haemostasis in factor V deficiency. Amer. J. med. Sci 245: 194 1964;
    PubMedSearch in Google Scholar
  • 51 Winckelmann G, Walther G. Zur Substitutionsbehandlung beim kongenitalen Faktor-V-Mangel. Blut 16: 345 1969;
    PubMedSearch in Google Scholar