Thromb Haemost 1959; 03(02): 237-256
DOI: 10.1055/s-0038-1654385
Originalarbeiten — Original Articles — Travaux Originaux
Schattauer GmbH

Prothrombin as Co-Factor of the Circulating Anticoagulant in Systemic Lupus Erythematosus?

A Loeliger
1   Clinic for Internal Medicine (Director: Prof. J. Mulder), University Hospital, Leyden, Holland
,
with the technical assistance of Miss,
E. J. J Alsbach
1   Clinic for Internal Medicine (Director: Prof. J. Mulder), University Hospital, Leyden, Holland
› Author Affiliations
We wish to thank colleague G. J. Sas, specialist in internal medicine in Dordrecht, for kindly putting his data at our disposal.
Further Information

Publication History

Publication Date:
08 June 2018 (online)

 

Summary

A 35-year old man with lupus erythematosus of the skin is demonstrated, who also shows signs of systemic L. E.: sometimes slightly increased ESR, varying thrombocytopenia, slightly prolonged coagulation and prothrombin time, positive cepnalin-cholesterol flocculation test, biologically false positive syphilis reactions and signs of a slightly increased haemolysis. Electrophoretic examination of the serum proteins presents practically normal values, certainly no hypergammaglobulinaemia. Analytical examination of coagulation reveals hypoprothrombinaemia and an anticoagulant most probably directed against thrombocyte factor 3, intrinsic thromboplastin and tissue thromboplastin. The anticoagulant seems to be only active in the presence of prothrombin. Prothrombin therefore, is considered a co-factor of the anticoagulant. The inactive anticoagulant is localized in the gamma globulins. The possible cause of the hypoprothrombinaemia (immune reaction?) and the data in the literature are discussed.


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  • References

  • 1 Barkhan P. Observations on a coagulation defect characterised by thrombocytopenia with a circulating anticoagulant. S. Afr. J. med. Sci 1952; 17: 87
  • 2 Biggs R, Macfarlane R. G. Human blood coagulation and its disorders. Black-well Scientific Publ; Oxford: 1957
  • 3 Bonnin J. A, Cohen A. K, Hicks N. D. Coagulation defects in a case of systemic lupus erythematosus with thrombocytopenia. Brit. J. Haematol 1956; 2: 168
  • 4 Conley C. L, Hartmann R. C. A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erytematosus. J. clin. Invest 1952; 31: 621
  • 5 Dameshek W. Systemic lupus erythematosus: A complex auto-immune disorder? Ann. internat. Med 1958; 48: 707
  • 6 AAAAAFrick P. G. Acquired circulating anticoagulants in systematic “collagen disease”. Blood 1955; 10: 691
  • 7 Hartert H. Klinische Blutgerinnungsstudien mit der Thrombelastographie. Dtsch. Arch. klin. Med 1952; 199: 284 (293, 402, 414)
  • 8 van Herwerden M. A. Een eenvoudige telmethode voor bloedplaatjes. Ned. T. Geneesk 1915; 11: 1866
  • 9 Hicks N. D, Pitney W. R. A rapid screening test for disorders of thromboplastin generation. Brit. J. Haematol 1957; 3: 227
  • 10 Hitzig W. H, Labhart A, Uehlinger E. Transitorische Hemmkörperhämo-philie bei Rheumatismus. Helv. med. Acta 1951; 18: 410
  • 11 Kovács E. Steigerung der endogenen Antithrombokinase-Produktion bei Lungenembolie und Myokardinfarkt. Thromb. Diath. haem 1957; 1: 541
  • 12 Laurell A. B, Nilsson I. M. Hypergammaglobulinemia, circulating anticoagulant, and biologic false positive Wassermann reaction. J. Lab. clin. Med 1957; 49: 694
  • 13 Lee S. L. Laboratory studies in systemic lupus erythematosus. AMA Arch. Dermat 1956; 73: 313
  • 14 Lee S. L, Sanders M. A disorder of blood coagulation in systemic lupus erythematosus. J. clin. Invest 1955; 34: 1814
  • 15 Ley A. B, Reader G. G, Sorenson C. W, Overman R. S. Idiopathic hypoprothrombinemia associated with hemorrhagic diathesis and the effect of vitamin K. Blood 1951; 6: 740
  • 16 Loeliger A. Über den Nachweis eines neuen Blutgerinnungsfaktors: Faktor VIL Wien. Z. inn. Med 1952; 33: 169
  • 17 Loe liger A, Hers J. F. Ph. Chronic Antithrombinaemia (Antithrombin V) with haemorrhagic diathesis in a case of rheumatoid arthritis with hypergammaglobulinaemia. Thromb. Diath. haem 1957; 1: 499
  • 18 Mueller J. F, Ratnoff O, Heinle R. W. Observations on the characteristics on an unusual circulating anticoagulant. J. Lab. clin. Med 1951; 38: 254
  • 19 Owren P. A, Aas K. The control of dicumarol therapy and the quantitative determination of prothrombin and proconvertin. Scand. J. clin. Lab. Invest 1951; 3: 201
  • 20 vanderPol E. T, Kettenborg H. K, de Vries S. L. Een modificatie van de “thrombin generation test”. Ned. T. Geneesk 1956; 100: 1900
  • 21 Post C. R, den Ottolander G. J. H, Hoorweg P. G. Een familiale vorm van idiopathische hypoprothrombinaemie. Ned. T. Geneesk 1956; 100: 1981
  • 22 Quick A. J. Hemorrhagic diseases. Lea & Febiger; Philadelphia: 1957
  • 23 Ramot C, Singer K. An unusual circulating anticoagulant in systemic lupus erythematosus. Acta haemat. (Basel) 1956; 16: 158
  • 24 Seligmann M. Etudes immunologiques sur le lupus érythémateux disséminé. Rev. Franç. d’Etud. Clin. et Biol 1958; 3: 558
  • 25 Seligmann M, Milgrom F, Trefouel J. Mise en évidence par la fixation du complément de la réaction entre acide désoxyribonucléique et sérum de malades atteints de lupus érythémateux disséminé. C. R. Acad. Sci. (Paris) 1958; 245: 1472
  • 26 Swift S. A serum anticoagulant factor in systemic lupus erythematosus. AMA Arch. Dermat 1956; 74: 296
  • 27 Verstraete M, Vandenbroucke J. Occurence and mode of action of endogenous circulating anticoagulant. J. Lab, clin. Med 1956; 48: 673

  • References

  • 1 Barkhan P. Observations on a coagulation defect characterised by thrombocytopenia with a circulating anticoagulant. S. Afr. J. med. Sci 1952; 17: 87
  • 2 Biggs R, Macfarlane R. G. Human blood coagulation and its disorders. Black-well Scientific Publ; Oxford: 1957
  • 3 Bonnin J. A, Cohen A. K, Hicks N. D. Coagulation defects in a case of systemic lupus erythematosus with thrombocytopenia. Brit. J. Haematol 1956; 2: 168
  • 4 Conley C. L, Hartmann R. C. A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erytematosus. J. clin. Invest 1952; 31: 621
  • 5 Dameshek W. Systemic lupus erythematosus: A complex auto-immune disorder? Ann. internat. Med 1958; 48: 707
  • 6 AAAAAFrick P. G. Acquired circulating anticoagulants in systematic “collagen disease”. Blood 1955; 10: 691
  • 7 Hartert H. Klinische Blutgerinnungsstudien mit der Thrombelastographie. Dtsch. Arch. klin. Med 1952; 199: 284 (293, 402, 414)
  • 8 van Herwerden M. A. Een eenvoudige telmethode voor bloedplaatjes. Ned. T. Geneesk 1915; 11: 1866
  • 9 Hicks N. D, Pitney W. R. A rapid screening test for disorders of thromboplastin generation. Brit. J. Haematol 1957; 3: 227
  • 10 Hitzig W. H, Labhart A, Uehlinger E. Transitorische Hemmkörperhämo-philie bei Rheumatismus. Helv. med. Acta 1951; 18: 410
  • 11 Kovács E. Steigerung der endogenen Antithrombokinase-Produktion bei Lungenembolie und Myokardinfarkt. Thromb. Diath. haem 1957; 1: 541
  • 12 Laurell A. B, Nilsson I. M. Hypergammaglobulinemia, circulating anticoagulant, and biologic false positive Wassermann reaction. J. Lab. clin. Med 1957; 49: 694
  • 13 Lee S. L. Laboratory studies in systemic lupus erythematosus. AMA Arch. Dermat 1956; 73: 313
  • 14 Lee S. L, Sanders M. A disorder of blood coagulation in systemic lupus erythematosus. J. clin. Invest 1955; 34: 1814
  • 15 Ley A. B, Reader G. G, Sorenson C. W, Overman R. S. Idiopathic hypoprothrombinemia associated with hemorrhagic diathesis and the effect of vitamin K. Blood 1951; 6: 740
  • 16 Loeliger A. Über den Nachweis eines neuen Blutgerinnungsfaktors: Faktor VIL Wien. Z. inn. Med 1952; 33: 169
  • 17 Loe liger A, Hers J. F. Ph. Chronic Antithrombinaemia (Antithrombin V) with haemorrhagic diathesis in a case of rheumatoid arthritis with hypergammaglobulinaemia. Thromb. Diath. haem 1957; 1: 499
  • 18 Mueller J. F, Ratnoff O, Heinle R. W. Observations on the characteristics on an unusual circulating anticoagulant. J. Lab. clin. Med 1951; 38: 254
  • 19 Owren P. A, Aas K. The control of dicumarol therapy and the quantitative determination of prothrombin and proconvertin. Scand. J. clin. Lab. Invest 1951; 3: 201
  • 20 vanderPol E. T, Kettenborg H. K, de Vries S. L. Een modificatie van de “thrombin generation test”. Ned. T. Geneesk 1956; 100: 1900
  • 21 Post C. R, den Ottolander G. J. H, Hoorweg P. G. Een familiale vorm van idiopathische hypoprothrombinaemie. Ned. T. Geneesk 1956; 100: 1981
  • 22 Quick A. J. Hemorrhagic diseases. Lea & Febiger; Philadelphia: 1957
  • 23 Ramot C, Singer K. An unusual circulating anticoagulant in systemic lupus erythematosus. Acta haemat. (Basel) 1956; 16: 158
  • 24 Seligmann M. Etudes immunologiques sur le lupus érythémateux disséminé. Rev. Franç. d’Etud. Clin. et Biol 1958; 3: 558
  • 25 Seligmann M, Milgrom F, Trefouel J. Mise en évidence par la fixation du complément de la réaction entre acide désoxyribonucléique et sérum de malades atteints de lupus érythémateux disséminé. C. R. Acad. Sci. (Paris) 1958; 245: 1472
  • 26 Swift S. A serum anticoagulant factor in systemic lupus erythematosus. AMA Arch. Dermat 1956; 74: 296
  • 27 Verstraete M, Vandenbroucke J. Occurence and mode of action of endogenous circulating anticoagulant. J. Lab, clin. Med 1956; 48: 673