Cryofibrinogenemia: A Marker of Severity of Cryoglobulinemic Vasculitis

Michaud, Martin; Moulis, Guillaume; Puissant, Bénédicte; Balardy, L.; Huart, Antoine; Gaches, F.; Cougoul, P.; Arlet, P.; Blancher, Antoine; Pourrat, Jacques; Sailler, L.

doi:10.1016/j.amjmed.2015.03.009

Cited by 16 publications

(6 citation statements)

References 16 publications

(16 reference statements)

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“…Over time, various clinical tests for SF were developed and its blood levels were also found elevated in inflammation by numerous reports. For example, elevated SF levels (also known as Cryofibrinogenemia) were found in 8-13% of hospitalized patients that included infection, malignancy, and autoimmune disorders such as severe cryoglobulinemic vasculitis (Michaud et al 2015). Increased SF was also demonstrated in 12% of a large group of cryoglobulinemic patients (Saadoun et al 2009).…”

Section: Introductionmentioning

confidence: 99%

Novel characteristics of soluble fibrin: hypercoagulability and acceleration of blood sedimentation rate mediated by its generation of erythrocyte-linked fibers

et al. 2022

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Soluble fibrin (SF) in blood consists of monomers lacking both fibrinopeptides A with a minor population in multimeric clusters. It is a substantial component of isolated fibrinogen (fg), which spontaneously self-assembles into protofibrils progressing to fibers at sub-physiologic temperatures, a process enhanced by adsorption to hydrophobic and some metal surfaces. Comparisons of SF-rich (FR) and SF-depleted (FD) fg isolates disclosed distinct molecular imprints of each via an adsorption/desorption procedure using gold surfaced silica microplates. Accelerated plasminogen activator-induced lysis and decreased stiffness (G′) of thrombin-induced FR fg clots were revealed by thomboelastography. Erythrocyte sedimentation (ESR) in afibrinogenemic plasma (Hematocrit 25–33%) was accelerated by FR fg nearly threefold that of FD fg. Stained smears disclosed frequent rouleaux formations and fibers linking stacked erythrocytes in contrast to no rouleaux by FD fg. Rouleaux formations were more pronounced at 4 °C than at ambient temperatures and at fiber-membrane contacts displayed irregular, knobby membrane contours. One of several FR fg isolates also displayed incomplete fiber networks in cell-free areas. What is more, pre-mixing FR fg with each of three monoclonal IgG anti-fg antibodies at 1.5 mol/mol fg, that inhibited fibrin polymerization, prevented rouleaux formation save occasional 2–4 erythrocyte aggregates. We conclude that spontaneously generated SF fibers bound to erythrocytes forming intercellular links culminating in rouleaux formation and ensuing ESR acceleration which in clinical settings reflects hypercoagulability. Also, the results can explain the reported fg binding to erythrocytes via ligands such as CD47, stable in vivo RBC aggregates in capillaries, and red areas of pathologic thrombi.

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Section: Introductionmentioning

confidence: 99%

Novel characteristics of soluble fibrin: hypercoagulability and acceleration of blood sedimentation rate mediated by its generation of erythrocyte-linked fibers

et al. 2022

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“…Of protease inhibitors, alpha-1-antitrypsin and alpha-2-macroglobulin inhibit plasmin 7. In our case, awareness to the hatching of neoplastic pathology is recommended.…”

Section: Discussionmentioning

confidence: 82%

“…CF can be associated with serious conditions (autoimmune disease, infections and cancer) or isolated 6. In a single-centre retrospective study by Michaud et al 7 that included 224 patients with cryoglobulinaemic vasculitis who were also tested for CF, only 18 patients were found positive for both cryoglobulin and cryofibrinogen. In the CF subgroup, type III CF was the most frequent laboratory feature (50%).…”

Section: Discussionmentioning

confidence: 99%

Rare cause of bilateral foot gangrene: coexisting essential cryofibrogenaemia and cryoglobulinaemic vasculitis

Belfeki

Abroug²,

Strazzulla³

et al. 2019

BMJ Case Rep

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Cryofibrinogenaemia is a rare haematological disorder characterised by cold temperature-induced precipitation of plasma proteins causing small-vessel occlusive vascular disorder with a hallmark of skin ulceration. It remains an underdiagnosed entity because of a lack of diagnostic criteria. Cryoglobulinaemia vasculitis is a small-vessel vasculitis involving the skin, the joints, the peripheral nerve system and the kidneys. Its association with cryofibrinogenaemia causes more severe phenotype with poor prognosis. We describe the case of a 59-year-old woman presenting with cold-induced extensive bilateral foot gangrene due to coexisting cryofibrinogenaemia and cryoglobulinaemic vasculitis that required bilateral amputation and rituximab perfusions as maintenance therapy.

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“…4 The finding herein that fibronectin was present in the most concentrated CF might represent an important element for the diagnosis of thrombotic events associated with CF. 17,22 Repeated detection of CF is interesting to evaluate its permanent or transitory presence. Herein in approximately 16% of samples, CF was transient, not systematically found in two or more consecutive samples with the limitation that there were only approximately 11% re-tested patients.…”

Section: Discussionmentioning

confidence: 99%

“…For CF characterization (adapted from Michaud et al 17 ) antifibrinogen (rabbit anti-human fibrinogen Ab, Dako, Glostrup, Denmark) and antifibronectin (rabbit anti-human fibronectin Ab, Siemens Healthcare, Marburg, Germany) antisera were used on EIF. In CF-positive samples, fibrinogen was quantified by radial immunodiffusion (RID), technique adapted for low concentrations of fibrinogen 18 (NOR-Partigen, Fibrinogen, Siemens Healthcare).…”

Section: Methodsmentioning

confidence: 99%

Cryofibrinogen—Characteristics and Association with Cryoglobulin: A Retrospective Study Out of a Series of 1,712 Samples over 7 Years

Kolopp

Miossec

2023

Thromb Haemost

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Background and objective: Cryofibrinogens (CF) and cryoglobulins (CG) are cryoproteins responsible for obstructive vasculopathy and vasculitis. The aim of this study was to compare the characteristics of CF and CG, and define the conditions of their association. Methods and results: This retrospective study was conducted at the Lyon University Hospitals, and included patients with at least one sample tested for CF and/or CG between September 2013 and April 2021. Serum and plasma samples were analyzed in very strict conditions of temperature. After cold precipitation, CF and CG were characterized and quantified in the cryoprecipitates. CRP and plasma fibrinogen levels were also investigated. Over this 7-year period, 1,712 samples for CF detection and 25,650 samples for CG detection were sent to the laboratory. Simultaneous testing of CF and CG was performed in 1,453/1,712 samples (85%). CF was less often positive than CG (8.3% vs 13.5%, p<0.0001). In positive CF samples, CG was associated in 28.9% of cases. In CF, fibrinogen was associated with fibronectin in 98/142 (69%) samples, especially in high concentrated CF. CF concentration was independent of CRP and plasma fibrinogen concentrations. Conclusion: The simultaneous detection of CF and CG is essential for the diagnosis of vasculitis or thromboembolic events and their treatment.

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Cryofibrinogenemia: A Marker of Severity of Cryoglobulinemic Vasculitis

Cited by 16 publications

References 16 publications

Novel characteristics of soluble fibrin: hypercoagulability and acceleration of blood sedimentation rate mediated by its generation of erythrocyte-linked fibers

Novel characteristics of soluble fibrin: hypercoagulability and acceleration of blood sedimentation rate mediated by its generation of erythrocyte-linked fibers

Rare cause of bilateral foot gangrene: coexisting essential cryofibrogenaemia and cryoglobulinaemic vasculitis

Cryofibrinogen—Characteristics and Association with Cryoglobulin: A Retrospective Study Out of a Series of 1,712 Samples over 7 Years

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