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An elderly woman with light chain myeloma presented with prolonged epistaxis and extensive cutaneous haematomas: her kappa/lambda ratio was high at 395, her coagulation screen, thrombin and reptilase times were abnormal, her FV and FX were in the low range in the absence of specific inhibitors, her Clauss fibrinogen was low at 0.95 g/l but antigenic FNG was 1.58 g/l. The patient denied treatment and died of progressive renal failure. We wish to describe the unusual association of FX and FV deficiency co-existing with an acquired dysfibrinogenaemia.
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http://dx.doi.org/10.1097/MBC.0000000000001280 | DOI Listing |
Res Pract Thromb Haemost
July 2025
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Congenital fibrinogen deficiencies (CFDs) comprise rare inherited disorders characterized by quantitative (afibrinogenemia, hypofibrinogenemia) or qualitative (dysfibrinogenemia, hypodysfibrinogenemia) abnormalities of fibrinogen. While CFDs are typically associated with bleeding, a paradoxical risk of both arterial and venous thrombosis is being increasingly recognized. Proposed mechanisms include impaired thrombin clearance due to a lack of fibrin formation and structurally abnormal fibrin clots that promote thrombin release into the circulation or hinder fibrinolysis.
View Article and Find Full Text PDFBlood Coagul Fibrinolysis
April 2024
Dumfries & Galloway Royal Infirmary, Dumfries, UK.
Front Cardiovasc Med
January 2024
General Internal Medicine & Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, Padova University School of Medicine, Padova, Italy.
The most frequent haematological malignancy associated with acquired hypo/dysfibrinogenemia is multiple myeloma. We present an unusual case of severe haemorrhagic diathesis due to acquired hypofibrinogenemia in a patient with early T-cell precursor acute lymphoblastic leukaemia/lymphoma (ETP-ALL/LBL). A 57-year-old male was admitted to the General Internal Medicine Department of Padova University Hospital for acute massive haematomas of the left lower extremity associated with macrohaematuria.
View Article and Find Full Text PDFFront Med (Lausanne)
December 2023
Department of Medicine, Hemostaseology, University Hospital, Goethe University Frankfurt, Frankfurt, Germany.
Introduction: Inherited or acquired molecular abnormalities form a clinically heterogeneous group of fibrinogen disorders called dysfibrinogenaemia. Apart from a pediatric case report and in contrast to other clinical conditions, acquired dysfibrinogenaemia has not been previously reported in septic patients.
Methods: In an observational cohort study, 79 adult septic patients were investigated for the presence of acquired dysfibrinogenaemia at the time of their admission to the intensive care unit (ICU) of the University Hospital Frankfurt.
Hematol Oncol Clin North Am
December 2021
Department of Internal Medicine, Division of Hematology and Hematologic Malignancies, University of Utah, 2000 Circle Hope Drive, Room 4126, Salt Lake City, UT 84112, USA. Electronic address:
Fibrinogen plays a fundamental role in coagulation through its support for platelet aggregation and its conversion to fibrin. Fibrin stabilizes clots and serves as a scaffold and immune effector before being broken down by the fibrinolytic system. Given its importance, abnormalities in fibrin(ogen) and fibrinolysis result in a variety of disorders with hemorrhagic and thrombotic manifestations.
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