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Recurring thrombo-embolic accidents caused by family-related deficiency of the fibrinolysis system

Thrombophile Diathese bei familiärer Störung der Fibrinolyse

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Zusammenfassung

Es wird über eine Familie berichtet, in der bei drei Schwestern und bei der Mutter rezidivierende Thromboembolien auftraten. Gerinnungsphysiologische Untersuchungen ergaben normale Gerinnungsfaktoren, aber eine Erhöhung von Inhibitoren der Plasminogenaktivierung. Gleiche Befunde wurden bei zwei weiteren klinisch unauffälligen Schwestern erhoben, während ein Bruder und der Vater normales Verhalten zeigten. Bei den Personen mit erhöhter Inhibitoraktivität wurde auch eine gesteigerte Plättchenaggregation festgestellt, die als Sekundärphänomen angesehen wird. Es wird eine autosomalrezessive vererbte Störung angenommen, welche für die klinisch manifeste thrombophile Diathese bei einem Teil der Familienmitglieder verantwortlich ist. Die Ergebnisse weisen auf die Notwendigkeit der Untersuchung des fibrinolytischen Systems bei Patienten mit rezidivierenden Thromboembolien und ihren engsten Familienangehörigen hin.

Summary

Several members of the same family underwent examinations on the coagulation and fibrinolysis systems after recurring thrombo-embolic disease was observed in one of the members of the family. The results of biological examinations revealed normal coagulation factors, but a high level of plasminogen activation inhibitors accompanied by an increase in platelet aggregation was observed. These results are compared with the few similar cases reported by other authors. The exact part played in this particular case by the platelets and the inhibitor in the patient's predisposition to venous thrombosis and the spread of the disease is discussed. It would seem that the platelet abnormality merely accompanys the increase in inhibitors which is passed on by recessive autosomal transmission. The authors consider that in all cases of thrombosis, particularly recurring thrombosis, an investigation, comprising a study of the fibrinolysis factors and particularly the inhibitors, must be carried out on the patient's close family.

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Authors and Affiliations

  1. Hemostasis Laboratory, Regional Blood Transfusion Center, 9–11, rue Lionnois, F-54000, Nancy, France

    P. Alexandre, A. Larcan & M. E. Briquel

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  1. P. Alexandre
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  2. A. Larcan
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  3. M. E. Briquel
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Alexandre, P., Larcan, A. & Briquel, M.E. Recurring thrombo-embolic accidents caused by family-related deficiency of the fibrinolysis system. Blut 41, 437–444 (1980). https://doi.org/10.1007/BF01007768

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  • DOI: https://doi.org/10.1007/BF01007768

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