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Thromb Haemost 1986; 55(01): 131-135
DOI: 10.1055/s-0038-1661463
Original Article
Schattauer GmbH Stuttgart

Fibrinogen Milano II: A Congenital Dysfibrinogenaemia Associated with Juvenile Arterial and Venous Thrombosis

F Haverkate
1   The TNO Gaubius Institute for Cardiovascular Research, Leiden, The Netherlands
,
J Koopman
1   The TNO Gaubius Institute for Cardiovascular Research, Leiden, The Netherlands
,
C Kluft
1   The TNO Gaubius Institute for Cardiovascular Research, Leiden, The Netherlands
,
A D’Angelo
2   The A. Bianchi Bonomi Hemophilia and Thrombosis Centre and Institute of Internal Medicine, University of Milan, Italy
,
M Cattaneo
2   The A. Bianchi Bonomi Hemophilia and Thrombosis Centre and Institute of Internal Medicine, University of Milan, Italy
,
P M Mannucci
2   The A. Bianchi Bonomi Hemophilia and Thrombosis Centre and Institute of Internal Medicine, University of Milan, Italy
› Author Affiliations
Further Information

Publication History

Received 12 September 1985

Accepted 06 December 1985

Publication Date:
19 July 2018 (online)

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Summary

A congenitally abnormal fibrinogen was isolated from blood of a young man with deep-vein thrombosis. Two other affected members of his family had three episodes of severe arterial thrombosis. The fibrinogen showed a delayed clotting by thrombin, but a normal clotting by Arvin®, Reptilase®, and prothrom-bin-staphylocoagulase complex. Analysis of the fibrinopeptides A and B by High Performance Liquid Chromatography did not reveal an abnormal peptide structure. The rate of release of A and B peptides by thrombin was strongly delayed, whereas the rate of release of fibrinopeptide A by Arvin® appeared to be normal. The fibrin polymerization rate was normal. Interactions between the abnormal fibrinogen, platelets and the fibrinolytic system were also normal. Evidence is presented that the defective interaction between fibrinogen Milano II and thrombin is associated with a defective binding of thrombin to the fibrin moiety of the abnormal fibrinogen.

Key words

Dysfibrinogenemia - Hereditary thrombosis Summary
 

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