eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Dysfibrinogenemia: Differential Diagnoses & Workup

Author: Wendy Brick, MD, Consulting Staff, Department of Internal Medicine, Division of Hematology and Oncology, Mecklenburg Medical Group
Coauthor(s): Russell Burgess, MD, Department of Internal Medicine, Division of Hematology/Oncology, East Carolina Internal Medicine; Guy B Faguet, MD, Former Professor, Department of Medicine, Section of Hematology and Oncology, Medical College of Georgia
Contributor Information and Disclosures

Updated: Nov 17, 2009

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
Further Reading
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References

  1. Acharya SS, Dimichele DM. Rare inherited disorders of fibrinogen. Haemophilia. Nov 2008;14(6):1151-8. [Medline].

  2. Kotlin R, Reicheltova Z, Maly M, et al. Two cases of congenital dysfibrinogenemia associated with thrombosis - Fibrinogen Praha III and Fibrinogen Plzen. Thromb Haemost. Sep 2009;102(3):479-86. [Medline].

  3. Morris TA, Marsh JJ, Chiles PG, et al. High prevalence of dysfibrinogenemia among patients with chronic thromboembolic pulmonary hypertension. Blood. Aug 27 2009;114(9):1929-36. [Medline].

  4. Miesbach W, Galanakis D, Scharrer I. Treatment of patients with dysfibrinogenemia and a history of abortions during pregnancy. Blood Coagul Fibrinolysis. Jul 2009;20(5):366-70. [Medline].

  5. 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].

  6. Galanakis DK. Inherited dysfibrinogenemia: emerging abnormal structure associations with pathologic and nonpathologic dysfunctions. Semin Thromb Hemost. 1993;19(4):386-95. [Medline].

  7. 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].

  8. Martinez J. Congenital dysfibrinogenemia. Curr Opin Hematol. Sep 1997;4(5):357-65. [Medline].

  9. 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.

  10. Mori T, Ikeda Y. [Acquired dysfibrinogenemia]. Ryoikibetsu Shokogun Shirizu. 1998;(21 Pt 2):529-31. [Medline].

  11. Mosesson MW. Dysfibrinogenemia and thrombosis. Semin Thromb Hemost. 1999;25(3):311-9. [Medline].

  12. 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.

  13. 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].

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Further Reading

Clinical guidelines:
Guidelines for the use of fresh-frozen plasma, cryoprecipitate and cryosupernatant. British Committee for Standards in Haematology - Professional Association. 2004 Jul. 18 pages. NGC:006191

Clinical trials:
Fibrinogen Concentrate (Human) − Efficacy and Safety Study

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Keywords

congenital dysfibrinogenemia, fibrinogen, fibrin, fibrinolysis, clotting cascade, coagulation factor, coagulation factors, clotting factor, clotting factors, coagulation disorders, coagulation disorder, abnormal clot formation, fibrinopeptide, thrombotic events

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Contributor Information and Disclosures

Author

Wendy Brick, MD, Consulting Staff, Department of Internal Medicine, Division of Hematology and Oncology, Mecklenburg Medical Group
Wendy Brick, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Medical Association, and American Society of Hematology
Disclosure: Nothing to disclose.

Coauthor(s)

Russell Burgess, MD, Department of Internal Medicine, Division of Hematology/Oncology, East Carolina Internal Medicine
Russell Burgess, MD is a member of the following medical societies: American College of Physicians and American Medical Association
Disclosure: Nothing to disclose.

Guy B Faguet, MD, Former Professor, Department of Medicine, Section of Hematology and Oncology, Medical College of Georgia
Guy B Faguet, MD is a member of the following medical societies: American Association of Immunologists, American Society of Hematology, International Society of Hematology, New York Academy of Sciences, Southern Medical Association, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

Medical Editor

Karen Seiter, MD, Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College
Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology
Disclosure: Novartis Honoraria Speaking and teaching; Schering Honoraria Speaking and teaching; Cephalon Honoraria Speaking and teaching

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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