Inherited Abnormalities of Fibrinogen Follow-up
- Author: Suchitra S Acharya, MBBS, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...
Further Outpatient Care
Ideally, individuals with afibrinogenemia and symptomatic dysfibrinogenemias should be monitored by a comprehensive bleeding disorder care team experienced in diagnosing and managing inherited bleeding disorders associated with a hemophilia and inherited bleeding disorders treatment center.
Individuals who may require plasma-derived coagulation factor concentrates should be immunized with the hepatitis A and hepatitis B vaccine.
Patients should avoid taking aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs (NSAIDs) and other drugs that affect platelet function.
Consider prophylactic therapy for patients with recurrent bleeding episodes, CNS hemorrhage, or during pregnancy for women with recurrent miscarriages.
Patients and families should be provided with instruction and educational materials to enhance understanding of their coagulation disorder, improve their ability to recognize the symptoms and signs of bleeding and/or thrombosis, and to identify emergency situations.
Patients should know how to contact their treatment center for immediate treatment and where to go to receive emergency care.
Patients should wear a MedicAlert bracelet or carry other identification of their hemostatic disorder and recommended therapy.
Bolton-Maggs PH, Perry DJ, Chalmers EA, et al. The rare coagulation disorders--review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation. Haemophilia. 2004 Sep. 10(5):593-628. [Medline].
Roberts HR, Stinchcombe TE, Gabriel DA. The dysfibrinogenaemias. Br J Haematol. 2001 Aug. 114(2):249-57. [Medline].
Asselta R, Duga S, Tenchini ML. The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost. 2006 Oct. 4(10):2115-29. [Medline].
Kirihara T, Fujikawa Y, Takeda W, Kurihara T, Sato K, Ueki T, et al. Congenital dysfibrinogenemia coincidentally diagnosed at the onset of chronic myelogenous leukemia. Rinsho Ketsueki. 2014 May. 55(5):541-5. [Medline].
Sumitha E, Jayandharan GR, Arora N, Abraham A, David S, Devi GS, et al. Molecular basis of quantitative fibrinogen disorders in 27 patients from India. Haemophilia. 2013 Jul. 19(4):611-8. [Medline].
Acharya SS, Coughlin A, Dimichele DM,. Rare Bleeding Disorder Registry: deficiencies of factors II, V, VII, X, XIII, fibrinogen and dysfibrinogenemias. J Thromb Haemost. 2004 Feb. 2(2):248-56. [Medline].
Haverkate F, Samama M. Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen. Thromb Haemost. 1995 Jan. 73(1):151-61. [Medline].
Kaur M, Kumar N, Bose SK, Rajendran A, Trehan A, Ahluwalia J. Congenital afibrinogenemia in a new born: a rare cause for bleeding. Blood Coagul Fibrinolysis. 2014 Feb 6. [Medline].
Peyvandi F, Haertel S, Knaub S, Mannucci PM. Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypofibrinogenemia. J Thromb Haemost. 2006 Jul. 4(7):1634-7. [Medline].
Acharya SS, Dimichele DM. Rare inherited disorders of fibrinogen. Haemophilia. 2008 Nov. 14(6):1151-8. [Medline].
Parameswaran R, Dickinson JP, de Lord S, et al. Spontaneous intracranial bleeding in two patients with congenital afibrinogenaemia and the role of replacement therapy. Haemophilia. 2000 Nov. 6(6):705-8. [Medline].
Martinez J. Congenital dysfibrinogenemia. Curr Opin Hematol. 1997 Sep. 4(5):357-65. [Medline].
Verhovsek M, Moffat KA, Hayward CP. Laboratory testing for fibrinogen abnormalities. Am J Hematol. 2008 Dec. 83(12):928-31. [Medline].
Van Cott EM, Smith EY, Galanakis DK. Elevated fibrinogen in an acute phase reaction prolongs the reptilase time but typically not the thrombin time. Am J Clin Pathol. 2002 Aug. 118(2):263-8. [Medline].
Cunningham MT, Brandt JT, Laposata M, Olson JD. Laboratory diagnosis of dysfibrinogenemia. Arch Pathol Lab Med. 2002. 126:499-505. [Medline].
Peyvandi F, Cattaneo M, Inbal A, De Moerloose P, Spreafico M. Rare bleeding disorders. Haemophilia. 2008 Jul. 14 Suppl 3:202-10. [Medline].
Manco-Johnson MJ, Dimichele D, Castaman G, Fremann S, Knaub S, Kalina U. Pharmacokinetics and safety of fibrinogen concentrate. J Thromb Haemost. 2009 Dec. 7(12):2064-9. [Medline].