Major Surgery in a Subject with Factor V Deficiency

E. J Melliger; F Duckert

doi:10.1055/s-0038-1654318

Thrombosis and Haemostasis, Inhaltsverzeichnis

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

¹Blood 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

¹Blood Coagulation and Fibrinolysis Laboratory (Head: PD Dr. F. Duckert) of the University Department of Medicine (Head: Prof. Dr. F. Koller), Bürgerspital, Basel ( Switzerland)

› Institutsangaben

Abstract

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.

Volltext

Referenzen

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;
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;
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;
4 Borchgrevink G. F, Owren P. A. Surgery in a patient with factor V (proaccelerin) deficiency. Acta med. scand 170: 743 1961;
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;
6 Brennan M. J, Monto R. W, Shafer H. G. Hemorrhagic diathesis due to Ac-globulin deficiency. Amer. J. clin. Path 22: 150 1952;
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;
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;
9 Brown Jr. I. W, Smith W. W, Howse R. M. Surgical management in hemophilia and the hemophiloid diseases. Ann. Surg 149: 721 1959;
10 Ghrobâk L, Bartos V, Brzek V, Hnizdovâ D. Coagulation properties of human thoracic duct lymph. Amer. J. med. Sci 253: 69 1967;
11 Goelho E, Verrastro T, Jamra M. Parahemofilia. A presentaçao de caso de deficiença de factor V. Rev. Hosp. Clin 12: 216 1957;
12 Deutsch E, Fleischhacker H. Zur Differentialdiagnose der Parahämophilie. Z. klin. Med 149: 493 1952;
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;
13a Duckert F. Le diagnostic des coagulopathies. Röntgen- und Laborpraxis 13: 156 171, 203, 224 1960;
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
15 Field J. B, Ware A. G. Studies on parahemophilia. J. clin. Invest 33: 932 1954;
16 Frank E, Bilhan N, Ekren H. Die Parahämophilie (Owren), eine neue Form der hämorrhagischen Diathese. Acta haemat. (Basel) 03: 70 1950;
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;
18 Gobbi F, Ascari E, Barbieri U. Deficit congenito di proaccelerina (paraemofilia di Owren). Minerva Med. (Torino) 51: 78 1960;
19 Heni F, Kraus I. Angeborener Faktor-Y-Mangel (Owren’sche Krankheit). Klin. Wschr 32: 633 1954;
20 Hjort P, Rapaport S. I, Owren P. A. Evidence that platelet accelerator (platelet factor I) is absorbed plasma proaccelerin. Blood 10: 1139 1955;
21 Hörder M. H. Isolierter Faktor-V-Mangel, bedingt durch einen spezifischen Hemmkörper. Acta haemat. (Basel) 13: 235 1955;
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;
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;
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;
25 Kagami M, Morita H. Parahaemophilia, the report of one family from Japan. Acta haemat. (Basel) 35: 102 1966;
25a Kappeler R. Das Verhalten von Faktor V im Serum unter normalen und pathologischen Bedingungen. Z. klin. Med 153: 103 1955;
26 Kingsley G. S. Familial factor V deficiency; the pattern of heredity. Quart. J. Med. N.S 23: 323 1954;
27 Kwa S. B, Chen Y. F. Congenital factor V deficiency — report of a case. Singapore med. J 06: 184 1965;
28 Larrieu M. J, Caen J, Grenet P, Gayla J, Bernard J. Hypoaccélérinémie congénitale (parahémophilie d’Owren). Sang 27: 117 1956;
29 Lewis H. J, Ferguson J. H. Hypoproaccelerinemia. Blood 10: 351 1955;
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;
31 Lopez V, Pflugshaupt R, Wirthner H, Bütler R. Hereditärer Faktor-V-Mangel (Parahämophilie) in einer Schweizer Familie. Schweiz, med. Wschr 99: 1354 1969;
32 Marx R. Konstitutionelle Pseudohypoprothrombinämien. Aerztl. Forsch 04: 567 1950;
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;
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;
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;
36 Outeirino JHernandez, Fayos JSânchez, Paniagua G, Avendano LHernando, Jaca MAguirre. Parahemophilia defecto congenito de factor V. Rev. clin. esp 101: 56 1966;
37 Owen Jr. C. A, Cooper T. Parahemophilia. Arch, intern. Med 95: 194 1955;
38 Owren P. A. Parahemophilia. Haemorrhagic diathesis due to absence of a previously unknown clotting factor. Lancet I: 446 1947;
39 Philips L. L, Little W. A. Factor V deficiency in obstetrics. Obst. Gynecol 19: 507 1962;
40 Redner B, Scalettar H, Weiner M. Parahemophilia (Owren’s disease). Pediatrics 12: 5 1953;
41 Rush B, Ellis H. The treatment of patients with factor V deficiency. Thrombos. Diathes. haemorrh. (Stuttg) 14: 74 1965;
42 Sacks M. S, Raccuglia G. Hereditary deficiency of proaccelerin (parahemophilia). A familial study. J. Lab. clin. Med 46: 98 1955;
43 Sailer S, Hinrichs R. Isolierter kongenitaler Faktor-V-Mangel als Ursache einer hämorrhagischen Diathese. Wien. Z. inn. Med 40: 10 1959;
44 Saint-Paul B, Chassaigne M. Déficit majeur en proaccélérine (Facteur V) d’origine congénitale « Maladie d’Owren ». Gaz. méd. France 73: 707 1966;
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;
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;
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;
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;
49 Tango M, Ujiie A, Ishikawa N, Yasukouchi T. Familial congenital factor V deficiency (parahemophilia). J. Jap. Soc. intern. Med 54: 816 1965;
50 Webster W. P, Roberts H. R, Penick G. D. Haemostasis in factor V deficiency. Amer. J. med. Sci 245: 194 1964;
51 Winckelmann G, Walther G. Zur Substitutionsbehandlung beim kongenitalen Faktor-V-Mangel. Blut 16: 345 1969;