Medication Summary
When patients experience bleeding, FFP or cryoprecipitate may be transfused, depending on the severity of the bleeding. Patients with recurrent thrombotic events may require long-term anticoagulation with Coumadin or subcutaneous heparin.
Clotting factor replacement therapies
Class Summary
These are used to replace the clotting factors needed when moderate-to-severe bleeding occurs. This most often occurs in acquired dysfibrinogenemias caused by a severely damaged liver that is unable to make clotting factors.[6]
Cryoprecipitate
Precipitate formed when FFP is thawed. Contains factor VIII, fibrinogen, vWF, and fibronectin. Primarily used to treat bleeding in patients with fibrinogen deficiencies or abnormalities.
Fresh frozen plasma
Plasma is the fluid compartment of blood containing the soluble clotting factors. Indications for using FFP include bleeding in patients with congenital coagulation defects and multiple coagulation factor deficiencies (severe liver disease).
Anticoagulants
Class Summary
Prevent recurrent or ongoing thromboembolic occlusion of the vertebrobasilar circulation.
Heparin
Used in patients with thrombotic tendencies who develop deep venous thrombosis, arterial thrombosis, or pulmonary embolism.
Warfarin (Coumadin)
Interferes with hepatic synthesis of vitamin K–dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders. Tailor dose to maintain an INR in the range of 2-3.
Enoxaparin (Lovenox)
Chronic subcutaneous therapy may be required in patients with recurrent thrombotic episodes.
Enhances inhibition of factor Xa and thrombin by increasing antithrombin III activity. In addition, preferentially increases inhibition of factor Xa.
Average duration of treatment is 7-14 d.
Acharya SS, Dimichele DM. Rare inherited disorders of fibrinogen. Haemophilia. Nov 2008;14(6):1151-8. [Medline].
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].
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].
Miesbach W, Schenk J, Alesci S, Lindhoff-Last E. Comparison of the fibrinogen Clauss assay and the fibrinogen PT derived method in patients with dysfibrinogenemia. Thromb Res. Dec 2010;126(6):e428-33. [Medline].
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].
Bornikova L, Peyvandi F, Allen G, Bernstein J, Manco-Johnson MJ. Fibrinogen replacement therapy for congenital fibrinogen deficiency. J Thromb Haemost. Sep 2011;9(9):1687-704. [Medline].
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. Congenital dysfibrinogenemia. Curr Opin Hematol. Sep 1997;4(5):357-65. [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.
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].

