title: Hyperfibrinogenolysis in disseminated adenocarcinoma.
abstract: We present the case of a 42-year-old woman with a recently acquired bleeding tendency. Coagulation studies and response to antifibrinolytic therapy suggested primary hyperfibrinogenolysis: markedly low levels of fibrinogen and alpha2-antiplasmin, normal levels of antithrombin III, protein C and protein S combined with an only borderline low number of platelets without evidence of microangiopathic haemolytic anaemia. A suspected causative adenocarcinoma of the lung was demonstrated. She was treated successfully with tranexamic acid and cryoprecipitate, until the tumor progressed and hyperfibrinogenolysis progressed to diffuse intravascular coagulation. Differential diagnosis of these coagulation disorders, with similar etiology, clinical and laboratory findings is reviewed. Therapeutic implications are discussed.
authors.affiliation: Division of Thrombosis, Haemostasis and Rheology, University Hospital Groningen, The Netherlands. k.meijer@int.azg.nl
authors.forename: K
authors.initials: K
authors.lastname: Meijer
authors.forename: W M
authors.initials: WM
authors.lastname: Smid
authors.forename: S
authors.initials: S
authors.lastname: Geerards
authors.forename: J
authors.initials: J
authors.lastname: van der Meer
mesh_terms: D000230:Adenocarcinoma / Q000150:complications* / Q000175:diagnosis; D000328:Adult; D000347:Afibrinogenemia / Q000175:diagnosis / Q000209:etiology*; D000933:Antifibrinolytic Agents / Q000627:therapeutic use*; D002545:Brain Ischemia / Q000209:etiology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005340:Fibrinogen / Q000378:metabolism*; D006474:Hemorrhagic Disorders / Q000097:blood / Q000209:etiology*; D006801:Humans; D008175:Lung Neoplasms / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D010257:Paraneoplastic Syndromes / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D014148:Tranexamic Acid / Q000627:therapeutic use*; D000979:alpha-2-Antiplasmin / Q000172:deficiency
chemical_list: D000933:Antifibrinolytic Agents; D005338:Fibrin Fibrinogen Degradation Products; D000979:alpha-2-Antiplasmin; C036309:fibrin fragment D; D014148:Tranexamic Acid; D005340:Fibrinogen