Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00035024.xml
Thromb Haemost 1988; 59(02): 277-280
DOI: 10.1055/s-0038-1642770
DOI: 10.1055/s-0038-1642770
Original Articles
Congenital Deficiency of Plasminogen and Its Relationship to Venous Thrombosis
Further Information
Publication History
Received 16 June 1987
Accepted after revision 21 December 1987
Publication Date:
21 May 2018 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00035024/198802/lookinside/thumbnails/10-1055-s-0038-1642770-1.jpg)
Summary
In a patient with deep venous thrombosis, plasma concentrations of coagulant and inhibitor proteins were normal except for moderate deficiency of plasminogen. Family studies revealed a similar deficiency in the mother and sister of the propositus. Evaluation of purified plasminogen demonstrated that it functioned normally. The patient represents our only example of plasminogen deficiency in 435 German individuals evaluated with a history of thromboembolism.
-
References
- 1 Egeberg O. Inherited antithrombin deficiency causing thrombophilia. Thromb Diath Haemorrh 1965; 13: 516-530
- 2 Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 1981; 68: 1370-1373
- 3 Comp PC, Nixon RR, Cooper MR, Esmon CT. Familial protein S deficiency is associated with recurrent thrombosis. J Clin Invest 1984; 74: 2082-2088
- 4 Isacson S, Nilsson IM. Defective fibrinolysis in blood in vein walls in recurrent idiopathic venous thrombosis. Acta Chir Scand 1972; 138: 313-319
- 5 Wiman B, Ljundberg B, Chmielewska J, Urden G, Blomback M, Johnsson H. The role of the fibrinolytic system in deep vein thrombosis. J Lab Clin Med 1985; 105: 265-270
- 6 Sas G, Blasko G, Banhegyi D, Jako J, Palos LA. Abnormal antithrombin III (Antithrombin Budapest) as a cause of familial thrombophilia. Thromb Diath Haemorrh 1974; 32: 105-115
- 7 Rodgers GM, Shuman MA. Congenital thrombotic disorders. Amer J Hematol 1986; 21: 419-430
- 8 Griffin JH, Bezeaud A, Evatt B, Mosher D. Functional and immunological studies of protein S in thromboembolic disease. Blood 1983; 62: 301-301 Abstr
- 9 Aoki N, Moroi M, Sakata Y, Yoshida N, Matsuda M. Abnormal plasminogen. A hereditary molecular abnormality found in a patient with recurrent thrombosis. J Clin Invest 1978; 61: 1186-1195
- 10 Wohl RC, Summaria L, Robbins KC. Physiological activation of the human fibrinolytic system. Isolation and characterization of human plasminogen variants, Chicago I and Chicago II. J Biol Chem 1979; 254: 9063-9069
- 11 Wohl RC, Summaria L, Chediak J, Rosenfeld S, Robbins KC. Human plasminogen variant Chicago III. Thromb Haemostas 1982; 48: 146-152
- 12 Miyata T, Iwanaga S, Sakata Y, Aoki N. Plasminogen Tochigi: Inactive plasmin from replacement of alanine-600 by threonine in the active site. Proc Natl Acad Scie USA 1982; 79: 6132-6136
- 13 Miyata T, Iwanaga S, Sakata Y, Aoki N, Takamatsu J, Kamiya T. Plasminogens Tochigi II and Nagoya. Two additional molecular defects with Ala-600-Thr replacement found in plasmin light chain variants. J Biochem 1984; 96: 277-287
- 14 Kazama M, Tahara C, Suzuki Z, Gohchi K, Abe T. Abnormal plasminogen, a case of recurrent thrombosis. Thromb Res 1981; 21: 517-22
- 15 Soria J, Soria C, Bertrand O, Dunn F, Drouet L, Caen JP. Plasminogen Paris I: Congenital abnormal plasminogen and its incidence to thrombosis. Thromb Res 1983; 32: 229-238
- 16 Sakata Y, Aoki N. Molecular abnormality of plasminogen. J Biol Chem 1980; 225: 5442-5447
- 17 Aoki N, Tateno K, Sakata Y. Differences of frequency distribution of plasminogen phenotypes between Japanese and American populations: New methods for the detection of plasminogen variants. Biochem Genet 1984; 22: 871-881
- 18 Scharrer IM, Wohl RC, Hach V, Sinio L, Boreisha I, Robbins KC. Investigation of a congenital abnormal plasminogen, Frankfurt I, and its relationship to thrombosis. Thromb Haemostas 1986; 55: 396-401
- 19 Hasegawa DK, Tyler BJ, Edson JR. Thrombotic disease in 3 families with inherited plasminogen deficiency. Blood Suppl (Suppl. 01) 1982; 60: 213a-213a Abstr
- 20 Ten Cate JW, Peters M, Biiller H. Isolated plasminogen deficiency in a patient with recurrent thromboembolic complications. Thromb Haemostas 1983; 50: 59-59 Abstr
- 21 Lottenberg R, Dolly FR, Kitchens CS. Recurring thromboembolic disease and pulmonary hypertension associated with severe hypoplas- minogenemia. Amer J Hematol 1985; 19: 181-193
- 22 Mannucci PM, Kluft C, Traas DW, Seveso P, D’Angelo A. Congenital plasminogen deficiency associated with venous thromboembolism: therapeutic trial with stanazol. Br J Haem 1986; 63: 753-759
- 23 Laurell CB. Quantitative estimation of protein by electrophoresis in agarose gel containing antibodies. Anal Biochem 1966; 15: 45-45
- 24 Friberger P, Knos M, Gustavsson S, Aurell L, Claeson G. Methods for determination of plasmin, antiplasmin and plasminogen by means of substrate S-2251. Haemostasis 1978; 7: 138-145
- 25 Lijnen HR, von Hoef B, Collen D. On the role of the carbohydrate side chains of human plasminogen in its interaction with alpha2-antiplasmin and fibrin. Eur J Biochem 1981; 120: 149-154
- 26 Peltz SW, Hardt TA, Mangel WF. Positive regulation of activation of plasminogen by urokinase: differences in Km for (glutamic acid)-plasminogen and lysine-plasminogen and effect of certain ω-aminoacids. Biochemistry 1982; 21: 2798-2804
- 27 Gram J, Jespersen J. A functional plasminogen assay utilizing the potentiating effect of fibrinogen to correct for the overestimation of plasminogen in pathological plasma samples. Thromb Haemostas 1985; 53: 255-259