eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders
Dysfibrinogenemia: Treatment & Medication
Updated: Nov 17, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- Medical treatment is not indicated in the majority of patients.
- Fresh frozen plasma (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.
- Administration of prophylactic cryoprecipitate may prevent recurrent miscarriages.
- Miesbach et al described the use of fibrinogen concentrates to avoid pregnancy loss in women with dysfibrinogenemia.4 (The obstetric complications of dysfibrinogenemia include first-trimester pregnancy loss, along with hemorrhage, placental abruption, and thrombosis.) The investigators performed a retrospective study of 4 women from the same family, each of whom had dysfibrinogenemia and a history of recurrent pregnancy loss. The patients received fibrinogen concentrates from the start of pregnancy until delivery, with 3 of the 4 women achieving delivery.
Consultations
Hematologist
Medication
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
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.
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.
Adult
1-4 U/10 kg IV (goal is measured fibrinogen >100 mg/dL)
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
A - Fetal risk not revealed in controlled studies in humans
Precautions
Viral contamination and infection are remotely possible but unlikely because of prescreening
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).
Adult
8-10 mL/kg IV
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
A - Fetal risk not revealed in controlled studies in humans
Precautions
Viral contamination and infection are possible but unlikely because of prescreening
Anticoagulants
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.
Adult
80 U/kg IV infusion initially, followed by 18 U/kg/h continuous infusion
Pediatric
Not established
Digoxin, nicotine, tetracycline, and antihistamines may decrease effects; NSAIDs, ASA, dextran, dipyridamole, and hydroxychloroquine may increase heparin toxicity
Documented hypersensitivity; severe thrombocytopenia with uncontrollable active bleeding; subacute bacterial endocarditis; history of heparin-induced thrombocytopenia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
In neonates, preservative-free heparin is recommended to avoid possible toxicity (gasping syndrome) from benzyl alcohol, which is used as preservative; caution in severe hypotension and shock; monitor for bleeding in peptic ulcer disease, menstruation, increased capillary permeability, and when administering IM injections
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.
Adult
5 mg PO qd initially; increase to desired INR
Pediatric
Not established
Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate; medications that may increase anticoagulant effects of warfarin include oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen and sulindac
Documented hypersensitivity; hemorrhagic tendencies; recent surgery of CNS or eye; traumatic surgery resulting in large open surfaces; overt bleeding of the GI tract, CNS, or aorta; threatened abortion; eclampsia or preeclampsia; unsupervised patients with senility, alcoholism, or psychosis
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients with protein C or S deficiency are at risk of developing skin necrosis; periodic determination of INR is essential
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.
Adult
1 mg/kg q12h SC injection (LMW heparin) enoxaparin
Pediatric
Not established
Platelet inhibitors or oral anticoagulants such as dipyridamole, salicylates, aspirin, NSAIDs, sulfinpyrazone, and ticlopidine may increase risk of bleeding
Documented hypersensitivity; major bleeding; thrombocytopenia
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
If thromboembolic event occurs despite LMWH prophylaxis, discontinue drug and initiate alternate therapy; elevation of hepatic transaminases may occur but is reversible; heparin-associated thrombocytopenia may occur with fractionated LMWH; 1 mg of protamine sulfate reverses the effect of approximately 1 mg of enoxaparin if significant bleeding complications develop
More on Dysfibrinogenemia |
| Overview: Dysfibrinogenemia |
| Differential Diagnoses & Workup: Dysfibrinogenemia |
 Treatment & Medication: Dysfibrinogenemia |
| Follow-up: Dysfibrinogenemia |
| References |
| Further Reading |
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References
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, 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].
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].
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
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
