dysfibrinogenemia


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dysfibrinogenemia

[‚dis·fī‚brin·ō·jə′nē·mē·ə]
(medicine)
The presence of abnormal fibrinogens in the blood.
References in periodicals archive ?
If the TT is also prolonged, then hypofibrinogenemia, dysfibrinogenemia, anticoagulant use (direct thrombin inhibitor), and exposure to bovine thrombin must be considered.
Objectives: Congenital dysfibrinogenemia is a rare hereditary disease characterized by normal antigen level but lower function level of fibrinogen.
Fibrinogen levels below 1g/L or 100mg/dL should be corrected with cryoprecipitate transfusion under the following circumstances: massive transfusion requirement, DIC, congenital dysfibrinogenemia or secondary to medications (Grade C, Level 2b).
There are major alterations in the hemostatic pathways in most patients with liver disease including altered platelet and endothelial function, altered clotting factors and conditions such as hyperfibrinolysis, dysfibrinogenemia and renal failure which may be superimposed on these underlying abnormalities.
These protein deficiencies/abnormalities are genetic and include entities such as antithrombin deficiency, protein C deficiency, protein S deficiency, Factor V Leiden mutation, prothrombin 20210, and dysfibrinogenemia, among others.
The differential diagnosis includes dysfibrinogenemia, prothrombin deficiency, factor V deficiency, combined deficiency of factors V and VIII (F5F8D), factor X deficiency, and hereditary combined deficiency of the vitamin K-dependent clotting factors.
This case report of a woman presenting with bleeding illustrates that laboratory tests can make a diagnosis of acquired dysfibrinogenemia when unsuspected clinically.
A normal reptilase time despite PTT >180 seconds (Table 1) eliminated dysfibrinogenemia as the cause of the coagulopathy and indicated the presence of heparin.
Other less common causes of inherited thrombophilia are the antithrombin III, protein C and S deficiencies, and rare conditions such as plasminogen and heparin cofactor-II deficiencies and dysfibrinogenemia.