Acquired causes of hypofibrinogenemia and dysfibrinoegenemia need to be ruled out. The most common cause of acquired dysfibrinogenemia is advanced liver disease.33 Fibrinogen abnormalities are observed in the majority of patients with cirrhosis, acute or chronic hepatitis, and in individuals with metastatic hepatoma34 or renal carcinoma.35 Acquired hypofibrinogenemia can occur with disseminated intravascular coagulation, and with L-asparaginase and valproate therapy.36,37{Gralnick, 1971 #5307;Hauser, 1996 #5308;Gralnick, 1971 #40}
Conditions Mimicking Congenital Fibrinogen Disorders
Fetal fibrinogen, which transitions to the adult form of the molecule approximately 3 weeks after birth,38 is characterized by increased sialic acid content and phosphorylation with decreased sulfation. These fetal posttranslational modifications result in a fibrin molecule that is more translucent and has reduced tensile strength compared to the adult fibrinogen form.
Fibrinogen synthesized in some individuals with liver disease also exhibits altered carbohydrate content, causing a condition mimicking dysfibrinogenemia in certain laboratory tests.34,39
Elevated fibrinogen levels due to an acute phase reaction can be associated with prolonged reptilase times, possibly due to increased sialation and/or phosphorylation.40
Conditions associated with a prolonged thrombin time and not related to fibrinogen deficiency include the following:
Rare Coagulation Disorders Resource Room, an extension of the RBDD Registry (eu.rbdd.org), was developed in collaboration with the Indiana Hemophilia & Thrombosis Center (www.innovativehematology.org).
The Resource Room serves as a platform to promote international collaboration to increase knowledge of these very rare disorders and to improve the care of affected individuals. The international RBDD mission, under the leadership of Dr. Peyvandi, will continue to be expanded over time.
The Resource Room thanks all contributing authors of the website articles that have made this valuable information available to the global community of both individuals affected with rare coagulation disorders and their healthcare providers.
