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Coagulation
Acquired bleeding disorders
Acquired dysfibrinogenemia
Reviewers: Kendall Crookston, M.D., Ph.D., University of New Mexico,
Lizabeth Rosenbaum, MD, University of New Mexico
Julie Gober-Wilcox, M.D., Resident, University of New Mexico (see Reviewers page)
Revised: 20 June 2010, last major update June 2010
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.
Definition
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● Abnormal fibrinogen molecule that causes a decrease in the rate of fibrin polymerization
● Rarely causes bleeding or thrombosis
Epidemiology
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● 80% prevalence in patients with liver disease
● 8% prevalence in patients with obstructive jaundice
Sites
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● Bleeding tends to be mucosal (menorrhagia) or into soft tissues
● Thromboses can be venous or arterial
Etiology
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● Usually caused by liver or biliary tract disease or acute phase reaction; also monoclonal immunoglobulin that binds to fibrinogen
● Abnormal fibrinogen has increased sialic acid residues, which increases the net negative charge of the molecule, promoting charge repulsion between fibrin monomers, leading to decreased fibrin polymerization
● In cancer-associated dysfibrinogenemia (hepatocellular carcinoma, cervical carcinoma, breast carcinoma, renal cell carcinoma), tumor cells may secrete abnormal fibrinogen
● Usually does not cause bleeding or thrombosis, but may in alcoholic liver disease
Clinical features
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● Patients are usually asymptomatic
● Rarely bleeding or thromboses
Laboratory
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● Screening tests include reptilase time and thrombin time
● Fibrinogen clotting activity/antigen ratio is confirmatory test
● Patients usually have abnormal liver function tests
● Should rule out dysfibrinogenemia in family members (i.e. rule out congenital form)
● Dysfibrinogenemia typically resolves if underlying disease improves (i.e. liver disease improves or cancer undergoes remission)
Laboratory testing algorithm
Prognostic factors
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● Difficult to assess as patients with liver disease often have coagulation defects that could contribute to bleeding or thrombosis
Case reports
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● 63 year old man with monoclonal light chain that binds fibrinogen (Haematologica 2007;92:e111)
● 72 year old man with myeloma paraprotein that interacts with fibrinogen (Acta Haematol 2008;120:75)
Treatment
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● Treat clinical findings (i.e. if patient is bleeding, can give cryoprecipitate; if patient has thrombosis, can give heparin followed by oral anticoagulants)
Differential Diagnosis
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● Congenital dysfibrinogenemia
● Autoantibodies against fibrinogen
Additional references
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● Arch Pathol Lab Med 2002;126:499, eMedicine
End of Coagulation > Acquired Bleeding disorders > Acquired dysfibrinogenemia
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