Hereditary defects in fibrinogen structure may result in a dysfunctional molecule with impaired formation of a fibrin clot.
Inheritance: variable and influenced by consanguinity. Severely affected patients are usually homozygous.
(1) arterial and/or venous thrombosis
(2) delayed wound healing or wound dehiscence
(3) recurrent spontaneous abortions (in pregnant females)
(4) bleeding disorder after trauma or surgery
Some patients are asymptomatic and are detected on laboratory testing.
(1) prolonged PT and aPTT
(2) prolonged thrombin time
(3) levels of fibrinogen may be low or normal
(4) formation of an abnormal fibrin clot
(5) soluble fibrinogen molecules may be detected as fibrin split products
(6) typically no deficiency in other coagulation factors can be demonstrated
(7) correction of defect by addition of normal plasma (no inhibitor)
An hereditary disorder of fibrinogen with low plasma levels and a dysfunctional molecule is termed congenital hypodysfibrinogenemia.
It is necessary to exclude other fibrinogen disorders:
(1) congenital afibrinogenemia
(2) acquired dysfibrinogenemia
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Specialty: Hematology Oncology, Clinical Laboratory