Congenital Deficiency of Plasminogen and Its Relationship to Venous Thrombosis

V Hach-Wunderle; I Scharrer; R Lottenberg

doi:10.1055/s-0038-1642770

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 1988; 59(02): 277-280
DOI: 10.1055/s-0038-1642770

Original Articles

Schattauer GmbH Stuttgart

Congenital Deficiency of Plasminogen and Its Relationship to Venous Thrombosis

V Hach-Wunderle

¹The Department of Internal Medicine, University Hospital, Frankfurt/Main, FRG

,

I Scharrer

¹The Department of Internal Medicine, University Hospital, Frankfurt/Main, FRG

,

R Lottenberg

²The Department of Medicine, College of Medicine, University of Florida, Gainesville, Florida, USA

› Institutsangaben

Abstract

als PDF herunterladen

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.

Keywords

Deep vein thrombosis - Deficiency of plasminogen - Hypercoagulability - Hypoplasminogenemia

PDF (513 kb)

Referenzen

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