Coagulation
Acquired bleeding disorders
Acquired dysfibrinogenemia
Last author update: 1 June 2010
Last staff update: 10 September 2020
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Acquired dysfibrinogenemia [title]
Table of Contents
Definition / general | Epidemiology | Etiology | Clinical features | Laboratory | Prognostic factors | Case reports | Treatment | Differential diagnosis | Additional referencesCite this page: Crookston K., Rosenbaum L., Gober-Wilcox J. Acquired dysfibrinogenemia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/coagulationacquireddysfibrinogenemia.html. Accessed April 24th, 2024.
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
- 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
- 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
- Autoantibodies against fibrinogen
- Congenital dysfibrinogenemia
Additional references
