Coagulation
Acquired bleeding disorders
Acquired dysfibrinogenemia

Authors: Kendall Crookston, M.D., Ph.D., Lizabeth Rosenbaum, M.D. and Julie Gober-Wilcox, M.D. (see Authors page)

Revised: 2 March 2016, last major update June 2010

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Acquired dysfibrinogenemia [title]
Cite this page: Acquired dysfibrinogenemia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/coagulationacquireddysfibrinogenemia.html. Accessed December 5th, 2016.
Definition / General
  • Abnormal fibrinogen molecule that causes a decrease in the rate of fibrin polymerization
  • Rarely causes bleeding or thrombosis
Epidemiology
  • 80% prevalence in patients with liver disease
  • 8% prevalence in patients with obstructive jaundice
Etiology
  • 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
  • Patients are usually asymptomatic
  • Rarely bleeding or thromboses
Laboratory
  • 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)
Prognostic Factors
  • Difficult to assess as patients with liver disease often have coagulation defects that could contribute to bleeding or thrombosis
Case Reports
Treatment
  • Treat clinical findings (i.e. if patient is bleeding, can give cryoprecipitate
  • If patient has thrombosis, can give heparin followed by oral anticoagulants)
Diagrams / Tables
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Laboratory testing algorithm

Differential Diagnosis
  • Autoantibodies against fibrinogen
  • Congenital dysfibrinogenemia