Hypofibrinogenemia Presenting as Intracranial Hemorrhage
Introduction: Factor I deficiency or fibrinogen deficiency, is a rare inherited bleeding disorder related to fibrinogen function in the blood coagulation cascade. They are classified into afibrinogenemia, hypofibrinogenemia and dysfibrinogenemia. Afibrinogenemia is defined as a lack of fibrinogen in the blood, i.e. <20 mg/dl of plasma with a frequency between 0.5 and 2 per million. Hypofibrinogenemia is partial deficiency of fibrinogen with levels of 20–80 mg/dl of plasma. Estimated frequency varies from 0.5 to 3 per million.
Case Report: We report regarding a 46 year old female patient, who presented with complaints of severe headache and right sided weakness. Examination revealed right Hemiplegia with BP of 144/90 mm Hg. Investigations revealed Creatine Kinase: 470IU/L, normal Blood counts, ESR and CRP. Thyroid profile was normal. Fibrinogen levels were 17.9mg/dl and repeat test after 1 week showed 24.6mg/dl. ECG showed incomplete left bundle branch block. Echocardiography was normal.
Conclusion: Hypofibrinogenemia rarely manifests as intracranial hemorrhage but, commonly presents with oral, mucosal and gastro intestinal tract bleeding. Patients admitted with stroke should be evaluated for fibrinogen deficiency and more emphasis to be laid in case of intracranial hemorrhage and previous history of bleeding.
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, 10(5):593-628
Vinod V Balasa, MD,http://emedicine.medscape.com/article/960677-overview#a0199
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, 2(2):248-256
Elliott BM, Aledort LM. Restoring hemostasis: fibrinogen concentrate versus Cryoprecipitate. Expert Rev Hematol. 2013, 6:277-286
Collen D, Tytgat GN, Claeys H, Piessens R. Metabolism and distribution of fibrinogen. I. Fibrinogen turnover in physiological conditions in humans. Br J Haematol. 1972, 22:681-700
Caroline Berube. Disorders of fibrinogen. Up to date Apr 2014
Peyvandi F, Haertel S, Knaub S, Mannucci PM. Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypofibrinogenemia. J Thromb Haemost. 2006, 4(7):1634-1637
Acharya SS, Dimichele DM. Rare inherited disorders of fibrinogen. Haemophilia. 2008, 14(6):1151-1158
- There are currently no refbacks.