eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders
Dysfibrinogenemia: Differential Diagnoses & Workup
Updated: Apr 13, 2006
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Differential Diagnoses
| Antiphospholipid Antibody Syndrome and
Pregnancy | Protein S Deficiency |
| Antiphospholipid Syndrome | von Willebrand Disease |
| Antithrombin Deficiency | |
| Hemophilia, Overview | |
| Protein C Deficiency |
Other Problems to Be Considered
Afibrinogenemia
Hypodysfibrinogenemia
Hypofibrinogenemia
Plasminogen deficiency
Factor V Leiden deficiency
Hyperhomocystinemia
Workup
Laboratory Studies
- Expect prothrombin time (PT) to be prolonged.
- Expect activated partial thromboplastin time (aPTT) to be prolonged.
- Thrombin time (TT) is the most sensitive screening test for dysfibrinogenemias. Expect TT to be prolonged in patients with bleeding tendencies. Shortened TT may occur in patients prone to thrombosis (fibrinogen Oslo I).
- Expect reptilase time to be prolonged.
- The fibrinogen level may be low, within the reference range, or high. However, a level within the reference range or a high level does not imply that the fibrinogen molecule functions appropriately. For this reason, assess both the clottable (functional) fibrinogen, which should be decreased, and the antigenic fibrinogen (detected only by immunoassay), which should be within the reference range. Definitive characterization of the abnormal fibrinogen can be performed in a research laboratory.
- Euglobulin clot lysis time may aid in the diagnosis. It is a crude measure of fibrinolytic potential. Elevate this value when the abnormal fibrinogen is more sensitive to lysis than usual.
More on Dysfibrinogenemia |
| Overview: Dysfibrinogenemia |
Differential Diagnoses & Workup: Dysfibrinogenemia |
| Treatment & Medication: Dysfibrinogenemia |
| Follow-up: Dysfibrinogenemia |
| References |
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References
Bazzan M, Tamponi G, Vaccarino A, et al. Natural and acquired inhibitors of hemostasis in selected symptomatic outpatients with venous thromboembolic disease. Haematologica. Jul-Aug 1997;82(4):420-2. [Medline].
Galanakis DK. Inherited dysfibrinogenemia: emerging abnormal structure associations with pathologic and nonpathologic dysfunctions. Semin Thromb Hemost. 1993;19(4):386-95. [Medline].
Haverkate F, Samama M. Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen. Thromb Haemost. Jan 1995;73(1):151-61. [Medline].
Martinez J. Quantitative and qualitative disorders of fibrinogen. In: Hoffman, et al, eds. Hematology: Basic Principles and Procedures. 2nd ed. Philadelphia, Pa: Churchill Livingstone;1995:1703-13, 2011-13.
Martinez J. Congenital dysfibrinogenemia. Curr Opin Hematol. Sep 1997;4(5):357-65. [Medline].
Mori T, Ikeda Y. [Acquired dysfibrinogenemia]. Ryoikibetsu Shokogun Shirizu. 1998;(21 Pt 2):529-31. [Medline].
Mosesson MW. Dysfibrinogenemia and thrombosis. Semin Thromb Hemost. 1999;25(3):311-9. [Medline].
Rodgers GM, Greenberg CS. Inherited coagulation disorders. In: Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, eds. Wintrobe's Clinical Hematology. 10th ed. Baltimore, Md: Williams & Wilkins;1999:1702-3.
Schorer AE, Singh J, Basara ML. Dysfibrinogenemia: a case with thrombosis (fibrinogen Richfield) and an overview of the clinical and laboratory spectrum. Am J Hematol. Nov 1995;50(3):200-8. [Medline].
Further Reading
Keywords
congenital dysfibrinogenemia, cirrhosis, hepatoma, hepatitis, abnormal clot formation, fibrinopeptide, fibrinolysis, thrombotic events, fibrin, dysfibrinogenemia of liver disease, acquired dysfibrinogenemia
Differential Diagnoses & Workup: Dysfibrinogenemia