Acquired Factor V Inhibitors in a Patient with End-stage Renal Disease

Kitazawa, Atsushi; Misawa, Hideo; Nagahori, Katsuhiro; Koda, Ryo; Yoshino, Atsunori; Kawamoto, Shinya; Takeda, Takeshi

doi:10.2169/internalmedicine.55.7369

Cited by 8 publications

(7 citation statements)

References 15 publications

(11 reference statements)

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“…In four of the above-mentioned patients, hemostatic defect in arteriovenous fistula may be related to antimicrobial therapy (Table 4 ) [ 3 , 9 – 16 ]. Furthermore, AiFVD and eosinophilia were reported in four other patients with renal disease beside our patient [ 12 , 17 – 19 ]. The above findings suggest that drug-induced immune response can cause AiFVD.…”

Section: Discussionsupporting

confidence: 63%

“…Hemostatic defect in arteriovenous fistula, which presents as persistent oozing and is a common complication in patients undergoing hemodialysis, was the first symptom in several of these reported cases. In four of the abovementioned patients, hemostatic defect in arteriovenous fistula may be related to antimicrobial therapy (Table 4) [3,[9][10][11][12][13][14][15][16]. Furthermore, AiFVD and eosinophilia were reported in four other patients with renal disease beside our patient [12,[17][18][19].…”

Section: Discussionmentioning

confidence: 69%

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Acquired factor V inhibitor with erythema and eosinophilia in a patient with end-stage renal disease

et al. 2022

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Autoimmune factor V deficiency (AiFVD) is a rare bleeding disorder caused by factor V inhibitors. In this report, we present the case of an 89-year-old man who developed bleeding tendency during surgery to create arteriovenous fistula for hemodialysis. The bleeding tendency developed with prolongation of activated partial thromboplastin and prothrombin time, following drug-induced eruption and eosinophilia. Significant reduction in coagulation factor activity and inhibitory pattern in cross-mixing tests suggested the presence of inhibitors to coagulation factors. Subsequently, we detected a factor V inhibitor and anti-factor V autoantibodies was confirmed using enzyme-linked immunosorbent assay with purified human plasma factor V. Thus, the patient was ‘definitely diagnosed’ with AiFVD in accordance with the diagnostic criteria enacted by the Japanese Ministry of Health, Labor, and Welfare. The bleeding tendency improved after initiating oral prednisolone 50 mg (1 mg/kg) followed by normalization of activated partial thromboplastin time and prothrombin time at the 34th day. After improving the coagulation system prolongation, the inhibitor and autoantibodies has been eradicated. Since it is suggested that drug-induced immune response can cause AiFVD, AiFVD should be considered in patients who undergo hemodialysis and develop failure of hemostasis and drug-induced eruption.

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Section: Discussionsupporting

confidence: 63%

Section: Discussionmentioning

confidence: 69%

Acquired factor V inhibitor with erythema and eosinophilia in a patient with end-stage renal disease

et al. 2022

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“…Both LA and anticardiolipin (or anti-β 2 -glycoprotein antibody) were positive in four patients which demonstrated isolated FV deficiency. [49][50][51][52] Their FV inhibitors disappeared shortly after initiation of steroid pulse therapy, administration of prednisolone, and absence of immunosuppressive therapy, respectively. Thus, the expression of FV inhibitor in these cases was unlikely caused by presence of an antiphospholipid antibody which is usually resistant to immunosuppressive therapies.…”

Section: Bleeding Sites/symptoms and Bleeding Severitymentioning

confidence: 99%

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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“…One case was undergoing haemodialysis as our patient and the two others have membranous nephropathy, suggesting an underlying immuno-allergic process [16,17].…”

Section: Discussionmentioning

confidence: 90%

Haemodialysis Tunisian patient with acquired factor V inhibitor associated to arteriovenous shunt thrombosis

Ghachem¹,

Borgi

Salem³

et al. 2020

Annales De Biologie Clinique

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Acquired Factor V Inhibitors in a Patient with End-stage Renal Disease

Cited by 8 publications

References 15 publications

Acquired factor V inhibitor with erythema and eosinophilia in a patient with end-stage renal disease

Acquired factor V inhibitor with erythema and eosinophilia in a patient with end-stage renal disease

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

Haemodialysis Tunisian patient with acquired factor V inhibitor associated to arteriovenous shunt thrombosis

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