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Acquired factor V and factor X Deficiency coexisting with acquired dysfibrinogenaemia in a patient with light chain myeloma.
Blood Coagul Fibrinolysis
April 2024
Dumfries & Galloway Royal Infirmary, Dumfries, UK.
Article Synopsis
- - An elderly woman with light chain myeloma experienced severe nosebleeds and significant bruising due to abnormal blood clotting factors and low fibrinogen levels.
- - Her lab results showed a very high kappa/lambda ratio, indicative of her myeloma, and abnormal results in various coagulation tests.
- - Despite the diagnosis, the patient declined treatment and ultimately passed away from progressive renal failure, highlighting the rare combination of Factor X and Factor V deficiencies alongside acquired dysfibrinogenemia.
Severe haemorrhagic diathesis due to acquired hypofibrinogenemia in a patient with early T-cell precursor acute lymphoblastic leukaemia/lymphoma: a case report.
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 PDFInvestigation of acquired dysfibrinogenaemia in adult patients with sepsis using fibrinogen function vs. concentration ratios: a cross-sectional study.
Front 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.
Disorders of Fibrinogen and Fibrinolysis.
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.
View Article and Find Full Text PDFAutomated screening procedure for the phenotypes of congenital fibrinogen disorders using novel parameters, |min1|c and Ac/|min1|c, obtained from clot waveform analysis using the Clauss method.
Clin Chim Acta
October 2021
Department of Clinical Laboratory Investigation, Graduate School of Medicine, Shinshu University, Matsumoto, Japan; Laboratory of Clinical Chemistry and Immunology, Department of Biomedical Laboratory Sciences, School of Health Sciences, Shinshu University, Matsumoto, Japan.
Introduction: Fibrinogen activity (Ac) is widely measured, but fibrinogen antigen (Ag) is measured only in specialized laboratories, so it is difficult to discriminate congenital fibrinogen disorders (CFDs) from acquired hypofibrinogenemia (aHypo). In this study, to screen for CFD phenotypes we adopted novel parameters, |min1|c and Ac/ |min1|c, and compared these with validated Ac, Ag, and Ac/Ag, and previously proposed Ac/dH and Ac/|min1|.
Materials And Methods: We calibrated |min1| using a CN-6000 instrument and investigated the correlation between Ag and |min1|c for aHypo (n = 131) and CFD [18 dysfibrinogenemia (Dys), two hypodysfibrinogenemia (Hypodys) and four hypofibrinpogenemia (Hypo)].
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