Use of Fibrinogen Determination Methods in Differential Diagnosis of Hypofibrinogenemia and Dysfibrinogenemia

Škorňová, Ingrid; Šimurda, Tomáš; Staško, J.; Horváth, Denis; Žolková, Jana; Holly, Pavol; Brunclíková, Monika; Samoš, Matej; Bolek, Tomáš; Schnierer, Martin; Slavík, Luděk; Kubisz, Peter

doi:10.7754/clin.lab.2020.200820

Cited by 9 publications

(13 citation statements)

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“…There may also be slight differences in the normal levels ranges according to gender, age, geographic region, race, or ethnic origin. Fibrinogen has a circulating half-life of 3-5 days [20][21][22][23][24].…”

Section: Structure and Function Of Fibrinogenmentioning

confidence: 99%

“…The most accurate method to determine fibrinogen level is the Clauss assay, based on the comparison of TT of dilutions of plasma against a plasma standard. PT-derived fibrinogen assay is not a direct determination of fibrinogen activity; measurement derives from the change in light transmission or scatter from a PT curve [22]. Assay for measuring the fibrinogen antigen is performed using luminescent immunoassay (LIA).…”

Section: Laboratory Assaysmentioning

confidence: 99%

“…Assay for measuring the fibrinogen antigen is performed using luminescent immunoassay (LIA). Immunological assays are based on the determination of antigen concentration using a specific antibody directed against fibrinogen: LIA, EID (immunoprecipitation method called electroimmunodiffusion), or ELISA (enzyme-linked immunosorbent assay) [1,22]. Global hemostasis tests are used in diagnostics in specialized laboratories to better determine the clinical phenotype (Table 2) [2].…”

Section: Laboratory Assaysmentioning

confidence: 99%

“…Most patients with congenital hypofibrinogenemia are incidentally diagnosed during routine coagulation screening [56]. The diagnosis of hypofibrinogenemia is based on the detection of a proportional reduction of both fibrinogen activity below the lower limit of the normal level (<1.8 g/L) [22]. Routine clotting times are prolonged in proportion to the fibrinogen deficiency.…”

Section: Laboratory Assaysmentioning

confidence: 99%

“…activity below the lower limit of the normal level (<1.8 g/L) [22]. Routine clotting times are prolonged in proportion to the fibrinogen deficiency.…”

Section: Laboratory Assaysmentioning

confidence: 99%

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Congenital Afibrinogenemia and Hypofibrinogenemia: Laboratory and Genetic Testing in Rare Bleeding Disorders with Life-Threatening Clinical Manifestations and Challenging Management

et al. 2021

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Congenital fibrinogen disorders are rare pathologies of the hemostasis, comprising quantitative (afibrinogenemia, hypofibrinogenemia) and qualitative (dysfibrinogenemia and hypodysfibrinogenemia) disorders. The clinical phenotype is highly heterogeneous, being associated with bleeding, thrombosis, or absence of symptoms. Afibrinogenemia and hypofibrinogenemia are the consequence of mutations in the homozygous, heterozygous, or compound heterozygous state in one of three genes encoding the fibrinogen chains, which can affect the synthesis, assembly, intracellular processing, stability, or secretion of fibrinogen. In addition to standard coagulation tests depending on the formation of fibrin, diagnostics also includes global coagulation assays, which are effective in monitoring the management of replacement therapy. Genetic testing is a key point for confirming the clinical diagnosis. The identification of the precise genetic mutations of congenital fibrinogen disorders is of value to permit early testing of other at risk persons and better understand the correlation between clinical phenotype and genotype. Management of patients with afibrinogenemia is particularly challenging since there are no data from evidence-based medicine studies. Fibrinogen concentrate is used to treat bleeding, whereas for the treatment of thrombotic complications, administered low-molecular-weight heparin is most often. This review deals with updated information about afibrinogenemia and hypofibrinogenemia, contributing to the early diagnosis and effective treatment of these disorders.

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Section: Structure and Function Of Fibrinogenmentioning

confidence: 99%

Section: Laboratory Assaysmentioning

confidence: 99%

Section: Laboratory Assaysmentioning

confidence: 99%

Section: Laboratory Assaysmentioning

confidence: 99%

“…activity below the lower limit of the normal level (<1.8 g/L) [22]. Routine clotting times are prolonged in proportion to the fibrinogen deficiency.…”

Section: Laboratory Assaysmentioning

confidence: 99%

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Congenital Afibrinogenemia and Hypofibrinogenemia: Laboratory and Genetic Testing in Rare Bleeding Disorders with Life-Threatening Clinical Manifestations and Challenging Management

et al. 2021

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Congenital Fibrinogen Disorders, Diagnosis, and Management

Casini

2023

Congenital Bleeding Disorders

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Development and validation of a novel qualitative test for plasma fibrinogen utilizing clot waveform analysis

Suzuki

Kanematsu

et al. 2022

Sci Rep

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Plasma fibrinogen is commonly examined by Clauss fibrinogen assay, which cannot distinguish between quantitative and qualitative fibrinogen anomalies. However, our previously reported Clauss fibrinogen assay utilizing clot waveform analysis (Clauss-CWA) provides additional information that contributes to the classification of fibrinogen anomalies. In this study, we adopted the Clauss-CWA method for an autoanalyzer to automatically measure the antigenic estimate (eAg) of fibrinogen in addition to the functional amount (Ac), and to thus provide the Ac/eAg ratio as a qualitative indicator. Performance was validated by receiver operating characteristics (ROC) and precision recall (PR) curve analyses using a patient cohort, consisting of a training cohort (n = 519) and a validation cohort (n = 523), both of which contained cases of congenital (hypo)dysfibrinogenemia as qualitative defects. We obtained an optimal cutoff of 0.65 for Ac/eAg by ROC curve analysis of the training cohort, offering superior sensitivity (> 0.9661) and specificity (1.000). This cutoff was validated in the validation cohort, providing positive predictive value > 0.933 and negative predictive value > 0.998. PR curve analysis also showed that Clauss-CWA provided excellent performance for detecting qualitative fibrinogen anomalies. The Clauss-CWA method may represent a useful approach for detecting qualitative fibrinogen abnormalities in routine laboratory testing.

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Use of Fibrinogen Determination Methods in Differential Diagnosis of Hypofibrinogenemia and Dysfibrinogenemia

Cited by 9 publications

References 0 publications

Congenital Afibrinogenemia and Hypofibrinogenemia: Laboratory and Genetic Testing in Rare Bleeding Disorders with Life-Threatening Clinical Manifestations and Challenging Management

Congenital Afibrinogenemia and Hypofibrinogenemia: Laboratory and Genetic Testing in Rare Bleeding Disorders with Life-Threatening Clinical Manifestations and Challenging Management

Congenital Fibrinogen Disorders, Diagnosis, and Management

Development and validation of a novel qualitative test for plasma fibrinogen utilizing clot waveform analysis

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