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Dusart syndrome: a new concept of the relationship between fibrin clot
architecture and fibrin clot degradability: hypofibrinolysis related to an
abnormal clot structure
JP Collet, J Soria, M Mirshahi, M Hirsch, FB Dagonnet, J Caen and C Soria
Geffacs, Laboratoire Sainte-Marie, Paris, France.
Fibrinogen Dusart is a congenital dysfibrinogenemia (A-alpha 554
Arginine-->Cysteine) associated with severe thrombotic disorder, high
incidence of thrombotic embolism, and abnormal fibrin polymerization. This
thrombotic disorder was attributed to an abnormal clot thrombolysis with
reduced plasminogen binding to fibrin and defective plasminogen activation
by tissue plasminogen activator. The purpose of this work was to assess
whether clot architecture could be involved in the thromboresistance of the
fibrin Dusart and the high incidence of embolism. An important change in
Dusart fibrin clot structure was identified with dramatic decrease of gel
porosity (Ks), fiber diameters (d), and fiber mass-length ratios (mu)
derived from permeation analysis. In addition, rigidity of the Dusart clot
was found to be greatly increased compared with normal fibrin. We provide
evidence that both thrombolysis resistance and abnormal rigidity of the
fibrin Dusart are related to this abnormal architecture, which impairs the
access of fibrinolytic enzymes to the fibrin and which is responsible for a
brittle clot that breaks easily, resulting in a high incidence of embolism.
Indeed, when restoring a normal clot structure by adding dextran 40 (30
mg/mL) before coagulation, clot thrombolysis and clot rigidity recovered
normal values. This effect was found to be dose- dependent. We conclude
that clot architecture is crucial for the propensity of blood clot to be
degraded and that abnormal clot structure can be highly thrombogenic in
vivo. The alpha-C domains of fibrinogen are determinant in fibrin clot
structure.
Volume 82,
Issue 8,
pp. 2462-2469,
10/15/1993
Copyright © 1993 by The American Society of Hematology
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