Thromb Haemost 1992; 68(06): 634-636
DOI: 10.1055/s-0038-1646334
Original Article
Schattauer GmbH Stuttgart

Treatment of Venous Thromboembolism in Patients with Congenital Deficiency of Antithrombin III

S Schulman
The Dept of Internal Medicine, Karolinska Hospital, Stockholm and Dept of Medicine II, Sahlgren’s Hospital, Gothenburg, Sweden
,
L Tengborn
The Dept of Internal Medicine, Karolinska Hospital, Stockholm and Dept of Medicine II, Sahlgren’s Hospital, Gothenburg, Sweden
› Institutsangaben
Weitere Informationen

Publikationsverlauf

Received 27. März 1992

Accepted after revision 31. Juli 1992

Publikationsdatum:
04. Juli 2018 (online)

Summary

The treatment course of all thromboembolic events in the patients with congenital deficiency of antithrombin III (AT III) in the national Swedish register was reviewed in order to assess the appropriate medical therapy in this situation. The medical treatment of 70 events of venous thromboembolism was evaluated. There were eight cases with signs of clinical progression. The risk of therapeutic failure with heparin could be as low as 1.5% or as high as 9.2%. It would not be cost-effective to substitute with concentrates of AT III in every case with congenital deficiency thereof in connection with acute venous thromboembolism. “Heparin resistance” does not seem to be a problem in the vast majority of these patients.

 
  • REFERENCES

  • 1 Gruenberg JC, Smallridge RC, Rosenberg RD. Inherited antithrombin-III deficiency causing mesenteric venous infarction; a new clinical entity. Ann Surg 1975; 181: 791-794
  • 2 Rey JL, Delobel J, Claisse JF, Quiret JC, Bor Ph, Bernasconi P. Embolie pulmonaire sévère et thrombophlébite récidivante en rapport avec un déficit héréditaire en antithrombine III. Arch Mal Cœur 1979; 72: 919-923
  • 3 Hofman KJ, Goldman AP, Lurie M, Hockly J, Bradlow BA. Familial thrombosis associated with antithrombin III deficiency in a young adult male. S Afr Med J 1980; 58: 531-533
  • 4 Schwartz RS, Bauer KA, Rosenberg RD. et al Clinical experience with antithrombin III concentrate in treatment of congenital and acquired deficiency of antithrombin. Am J Med 1989; 87 (Suppl 3 B): 53-60
  • 5 Menache D, O’Malley JP, Schorr JB, Wagner B, Williams C. the Cooperative Study Group Evaluation of the safety, recovery, half-life, and clinical efficacy of antithrombin III (human) in patients with hereditary antithrombin III deficiency. Blood 1990; 75: 33-39
  • 6 Marciniak E, Gockerman JP. Heparin-induced decrease in circulating antithrombin III. Lancet 1977; 2: 581-584
  • 7 Tengborn L. Behandling av akut tromboembolism vid hereditär antitrombinbrist - en översikt. Läkartidningen 1987; 84: 1249-1250
  • 8 Sas G. Classification of antithrombin III deficiencies – has a new tower of Babel been built. Thromb Haemostas 1988; 60: 530-531
  • 9 Bjarke B, Herin P, Blombäck M. Neonatal aortic thrombosis. A possible clinical manifestation of congenital antithrombin III deficiency. Acta Paediat Scand 1974; 63: 297-301
  • 10 Hellgren M, Tenborn L, Abildgaard U. Pregnancy in women with congenital antithrombin III deficiency: Experience of treatment with heparin and antithrombin. Gynecol Obstet Invest 1982; 14: 127-141
  • 11 Rosendaal FR, Heijboer H, Briët E. et al Mortality in hereditary antithrombin-III deficiency – 1830 to 1989. Lancet 1991; 337: 260-262
  • 12 Tengborn L, Bergqvist D. Surgery in patients with congenital antithrombin III deficiency. Acta Chir Scand 1988; 154: 179-183
  • 13 Tengborn L, Wallmark A, Olsson K, Heijkenskjöld A-M. Koagulations- och fibrinolysutredning av patienter med ökad trombosbenägenhet. Läkartidningen 1988; 85: 1608-1611
  • 14 Winter JH, Fenech A, Mackie M, Bennett B, Douglas AS. Treatment of venous thrombosis in antithrombin III deficient patients with concentrates of antithrombin III. Clin Lab Hematol 1982; 4: 101-108
  • 15 Laharrague P, Caussé B, Bouissou F, Boucays A, Boneu B. Déficits constitutionnels et acquis en antithrombine III. Rev Med Toulouse 1980; 16: 201-205
  • 16 Miller N, Hultin MB, Gounder M, Zarrabi MH. Hereditary antithrombin III deficiency: case report and review of recent therapeutic advances. Am J Hematol 1986; 21: 215-221
  • 17 Chamontin B, Guittard J, Laroche M, Boneu B, Salvador M. Traitement d’une thrombose veineuse profonde en présence d’un déficit congénital en antithrombine III. Arch Mal Cœur 1984; 77: 1064-1067