Transient Factor V deficiency associated with Factor V-immunoglobulin complexes but without evidence of a classical inhibitor

Cortier, D.; Dreden, Patrick Van; Adam, Miriam; Bironien, Roselyne; François, Déborah; Vasse, Marc

doi:10.1016/j.thromres.2016.09.013

Cited by 3 publications

(2 citation statements)

References 10 publications

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“…Among these patients, 34 had IgG class and two had IgA class anti-FV autoantibodies. One patient had anti-FV autoantibodies, although the sample did not show a positive result for FV inhibitor, 16,17 suggesting the presence of nonneutralizing autoantibodies against FV and/or FVIII-antigen-antibody complexes, as reported by Cortier et al 53 Similarly, another patient showed low FV:C level (5%) in absence of an FV inhibitor and negative mixing test (paper in preparation). All patients with suspected AiFVD should be immunologically tested for anti-FV autoantibodies since functional FV inhibitor assays are unable to detect nonneutralizing autoantibodies against FV.…”

Section: Anti-factor V Autoantibodiesmentioning

confidence: 79%

A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on Japan

Ichinose

Osaki

Souri

2021

Semin Thromb Hemost

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Coagulation factor V (or FV for the purpose of medical safety) is an essential cofactor of coagulation factor X in the common pathway of coagulation; severe FV deficiency leads to a bleeding tendency. Although both congenital and acquired FV deficiencies are widely recognized, FV deficiency also presents as an autoimmune disorder. A nationwide survey on autoimmune coagulation factor deficiencies (AiCFDs) conducted in Japan by our Japanese Collaborative Research Group identified 24 new patients with autoimmune FV deficiency (AiFVD) in the past 5 years. Furthermore, our extensive literature search confirmed that 177 AiFVD cases have been reported in previous articles published from Japan. Patients with AiFVD in Japan were predominantly men, with age similar to those with other AiCFDs. AiFVD was confirmed as a relatively mild type of bleeding diathesis, associated with lower mortality rate than that for AiFVD and other AiCFDs reported in previous studies. Patients with AiFVD had variable FV inhibitor titers and both neutralizing anti-FV autoantibodies and nonneutralizing counterparts. Although spontaneous resolution occurs in some patients, timely initiation of hemostatic and immunosuppressive therapies helps arrest the bleeding and eliminate anti-FV antibodies, resulting in a high cumulative recovery rate. Immunological anti-FV antibody detection is recommended to avoid missing AiFVD cases for the presence of nonneutralizing anti-FV autoantibodies. Further investigation is necessary to clarify the long-term prognosis and optimal management of AiFVD.

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Section: Anti-factor V Autoantibodiesmentioning

confidence: 79%

A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on Japan

Ichinose

Osaki

Souri

2021

Semin Thromb Hemost

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“…[1][2][3] Limited reports of AiFVD exist, and among them, only a few involve AiFVD due to non-neutralizing anti-factor V antibody (anti-FVAb). 4,5 Antifibrinolytic therapy for disseminated intravascular coagulation (DIC) is contraindicated in principle due to the risk of systemic thrombosis 6 ; however, there have been reports of significant improvement in bleeding symptoms in enhanced-fibrinolytic-type DIC 7 when tranexamic acid is administered in combination with anticoagulation therapy. 8 We experienced a case of inhibitor-negative, nonneutralizing, anti-FVAb-positive AiFVD combined with enhancedfibrinolytic-type DIC, and gluteal muscle hematoma.…”

mentioning

confidence: 99%

Autoimmune‐acquired coagulation factor V deficiency with hyperfibrinolytic disseminated intravascular coagulation

Kaneda,

Fukuno,

Minami

et al. 2023

Int J Lab Hematology

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Retrospective examination of coagulation parameters in 33 patients with autoimmune coagulation factor deficiencies in Japan: A single-center analysis

Osaki¹,

Souri²,

Ogawa³

et al. 2022

Thrombosis Research

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Transient Factor V deficiency associated with Factor V-immunoglobulin complexes but without evidence of a classical inhibitor

Cited by 3 publications

References 10 publications

A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on Japan

A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on Japan

Autoimmune‐acquired coagulation factor V deficiency with hyperfibrinolytic disseminated intravascular coagulation

Retrospective examination of coagulation parameters in 33 patients with autoimmune coagulation factor deficiencies in Japan: A single-center analysis

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