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Fibrinogen

Thrombin

+ b B Fibrin peptides

Spontaneous polymerization

Weak fibrin polymer

Figure 18.1 Thrombin's activity on fibrinogen, from fibrin monomer to Cross-linked fibrin polymer fibrin polymer. (stable fibrin clot)

Figure 18.1 Thrombin's activity on fibrinogen, from fibrin monomer to Cross-linked fibrin polymer fibrin polymer. (stable fibrin clot)

Dysfibrinogenemia

These fibrinogen disorders are autosomal dominant and are inherited homozygously and heterozygously. They produce a qualitative disorder of fibrinogen in which an amino acid substitution produces a functionally abnormal fibrinogen molecule. Although these disorders are an academic curiosity, named for the city in which the patient was discovered, they are infrequently associated with a bleeding tendency. A few are associated with thrombosis.4 Approximately 40 abnormal fibrinogens have been discovered. Because fibrin formation is affected by the abnormal fibrinogen molecule in dys-fibrinogenemia, most of the normal laboratory assessments for fibrinogen will be abnormal. The PT, aPTT, TT, and reptilase time will be increased. An immunologic assay of fibrinogen that measures the antigenic level of fibrinogen is normal. The clottable assay for quantitative fibrinogen is abnormal as this assay is dependent on the proper amount and proper functioning fibrinogen.

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