Mayo Test Catalog, (Sorted By Test Name) - Mayo Medical ...

FFIBR
91933
FIB
8484
clinically. It is important to note that there are rare disorders of hemostasis that are also associated with
mutations in the FGA gene. Patients with afibrinogenemia, hypofibrinogenemia, and dysfibrinogenemia
have all been reported to have mutations in FGA. Most dysfibrinogenemias are autosomal dominant
disorders; afibrinogenemia and hypofibrinogenemia are more often autosomal recessive disorders. In
general, truncating mutations in FGA result in afibrinogenemia and missense mutations are a common
cause of dysfibrinogenemia.
Useful For: Carrier testing of individuals with a family history of fibrinogen alpha-chain (FGA)
gene-related familial visceral amyloidosis Diagnostic confirmation of FGA-related familial visceral
amyloidosis when familial mutations have been previously identified
Interpretation: An interpretive report will be provided.
Reference Values:
An interpretive report will be provided.
Clinical References: 1. Benson MD: The hereditary amyloidoses. Best Pract Res Clin Rhematol
2003;17:909-927 2. Benson MD: Ostertag revisited: The inherited systemic amyloidoses without
neuropathy. Amyloid 2005;12(2):75-87 3. Asselta R, Duga S, Tenchini ML: The molecular basis of
quantitative fibrinogen disorders. Thromb Haemost 2006 Oct;4(10):2115-2129 4. Shiller SM, Dogan A,
Highsmith WE: Laboratory methods for the diagnosis of hereditary amyloidoses. In
Amyloidosis-Mechanisms and Prospects for Therapy. Edited by S Sarantseva. InTech 2011, pp101-120
Fibrinogen Antigen
Reference Values:
201-454 mg/dL
Interpretive note: Differentiation of congenital from acquired defects
of fibrinogen requires clinical correlation. Fibrinogen antigen data
should be compared with functional fibrinogen activity on the same
sample for evaluation of afibrinogenemia, hypofibrinogenemia and
dysfibrinogenemia.
Test Performed by: BloodCenter of Wisconsin
638 N. 18th Street
Milwaukee, WI 53233-2121
Fibrinogen, Plasma
Clinical Information: Fibrinogen, also known as factor I, is a plasma protein that can be
transformed by thrombin into a fibrin gel ("the clot"). Fibrinogen is synthesized in the liver and
circulates in the plasma as a disulfide-bonded dimer of 3 subunit chains. The biological half-life of
plasma fibrinogen is 3 to 5 days. An isolated deficiency of fibrinogen may be inherited as an autosomal
recessive trait (afibrinogenemia or hypofibrinogenemia) and is 1 of the rarest of the inherited
coagulation factor deficiencies. Acquired causes of decreased fibrinogen levels include: acute or
decompensated intravascular coagulation and fibrinolysis (disseminated intravascular coagulation
[DIC]), advanced liver disease, L-asparaginase therapy, and therapy with fibrinolytic agents (eg,
streptokinase, urokinase, tissue plasminogen activator). Fibrinogen function abnormalities,
dysfibrinogenemias, may be inherited (congenital) or acquired. Patients with dysfibrinogenemia are
generally asymptomatic. However, the congenital dysfibrinogenemias are more likely than the acquired
to be associated with bleeding or thrombotic disorders. While the dysfibrinogenemias are generally not
associated with clinically significant hemostasis problems, they characteristically produce a prolonged
thrombin time clotting test. Congenital dysfibrinogenemias usually are inherited as autosomal
codominant traits. Acquired dysfibrinogenemias mainly occur in association with liver disease (eg,
chronic hepatitis, hepatoma) or renal diseases (eg, chronic glomerulonephritis, hypernephroma) and
usually are associated with elevated fibrinogen levels. Fibrinogen is an acute phase reactant, so a
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