title: Hypercoagulability in a patient with hypodysfibrinogenemia: implications for clinical management.
abstract: Dysfibrinogenemia accounts for approximately 0.7% of thrombophilia in patients with venous thromboembolic disease. In 20% of these patients, plasma thrombophilic dysfibrinogen is below 1.0 mg/ml, defining hypodysfibrinogenemia. We describe a young female patient, in whom hypodysfibrinogenemia was the cause of several severe thromboembolic events which occurred even under oral anticoagulation monitored by a standard prothrombin time (PT) test. In this patient, the standard PT test according to Quick underestimated the plasma coagulability in vivo, presumably due to the low levels of dysfunctional fibrinogen as the substrate of the thromboplastin reagent. A PT test supplemented with bovine plasma fibrinogen (Thrombotest) revealed lower fibrinogen-independent international normalized ratio (INR) values in the proposita on oral anticoagulation compared to a control group with eufibrinogenemia. Monitoring therapy with the fibrinogen-independent Thrombotest secured safe anticoagulation in this patient. We suggest to consider PT tests with exogenous fibrinogen (e.g. Thrombotest) to monitor oral anticoagulation in the rare thrombophilic patients with hypodysfibrinogenemia.
authors.affiliation: Department of Hematology and Oncology, University of Göttingen, Göttingen, Germany. gwulf@med.uni-goettingen.de
authors.forename: G G
authors.initials: GG
authors.lastname: Wulf
authors.forename: M
authors.initials: M
authors.lastname: Unterhalt
authors.forename: A
authors.initials: A
authors.lastname: Buchwald
authors.forename: D
authors.initials: D
authors.lastname: Zenker
authors.forename: H
authors.initials: H
authors.lastname: Kreuzer
authors.forename: W
authors.initials: W
authors.lastname: Hiddemann
mesh_terms: D000328:Adult; D001780:Blood Coagulation Tests; D019468:Disease Management; D005260:Female; D015241:Fibrinogens, Abnormal / Q000378:metabolism*; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D019934:International Normalized Ratio; D010644:Phenprocoumon / Q000627:therapeutic use; D019851:Thrombophilia / Q000150:complications / Q000378:metabolism / Q000628:therapy*; D013927:Thrombosis / Q000209:etiology / Q000378:metabolism / Q000628:therapy
chemical_list: D015241:Fibrinogens, Abnormal; D006493:Heparin; D010644:Phenprocoumon