Acquired Factor V Inhibitor after Coronavirus Disease 2019 (COVID-19)

Chiurazzi, Federico; Tufano, Antonella; Esposito, Maria; D'Agostino, F; Casoria, Aniello; Capasso, F.; Minno, Giovanni Di

doi:10.1055/s-0041-1735452

Cited by 5 publications

(4 citation statements)

References 16 publications

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“…Infectious diseases (viral and bacterial), antimicrobial agents (especially β-lactams), blood transfusion, cancer, and autoimmune diseases are the most common triggers of AiFVD. In a recent study, AiFVD was reported after Coronavirus Disease 2019 infection [ 6 ]. However, many cases of AiFVD are idiopathic, and the disease mechanism is not clear [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

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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: Discussionmentioning

confidence: 99%

“…In a recent study, AiFVD was reported after Coronavirus Disease 2019 infection [6]. However, many cases of AiFVD are idiopathic, and the disease mechanism is not clear [7].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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“…Perhaps unsurprisingly, COVID-19 has been associated with the majority of autoimmune conditions described in the previous sections of this review. In addition to potential APS, COVID-19 has been associated with HITT and HIT-like syndrome [ 64 , 90 , 91 ], as well as with acquired hemophilia A [ 92 ] and also acquired FV deficiency [ 93 ]. Again, the difficulty here is identifying if COVID-19 contributes to the reported events (i.e., “actor”), or whether COVID-19 is simply coincidental (i.e., “bystander”).…”

Section: Autoantibodies Interfering With Hemostasis In Covid-19 and A...mentioning

confidence: 99%

Autoimmune Diseases Affecting Hemostasis: A Narrative Review

Favaloro

Pasalic

Lippi

2022

IJMS

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Hemostasis reflects a homeostatic mechanism that aims to balance out pro-coagulant and anti-coagulant forces to maintain blood flow within the circulation. Simplistically, a relative excess of procoagulant forces can lead to thrombosis, and a relative excess of anticoagulant forces can lead to bleeding. There are a wide variety of congenital disorders associated with bleeding or thrombosis. In addition, there exist a vast array of autoimmune diseases that can also lead to either bleeding or thrombosis. For example, autoantibodies generated against clotting factors can lead to bleeding, of which acquired hemophilia A is the most common. As another example, autoimmune-mediated antibodies against phospholipids can generate a prothrombotic milieu in a condition known as antiphospholipid (antibody) syndrome (APS). Moreover, there exist various autoimmunity promoting environments that can lead to a variety of antibodies that affect hemostasis. Coronavirus disease 2019 (COVID-19) represents perhaps the contemporary example of such a state, with potential development of a kaleidoscope of such antibodies that primarily drive thrombosis, but may also lead to bleeding on rarer occasions. We provide here a narrative review to discuss the interaction between various autoimmune diseases and hemostasis.

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“…The clinical manifestation of this coagulopathy ranges from asymptomatic laboratory findings to life-threatening bleeding. There is a range of diverse clinical conditions that have been shown to associate with the presence of acquired factor V inhibitors (AFVI), including exposure to topical bovine thrombin glue during surgical procedures [4, 8,16,24,27], infections [1,10,29] including COVID-19 [6,11] and HIV [7], use of antibiotics (including post-operative antibiotic usage) [15,34], malignancies [10, 18,35] (including multiple myeloma [3,5]), treatment with anti-cancer drugs [13], other drug treatments [14], autoimmune diseases [20], and transfusion. 20% of the AFVI cases are idiopathic.…”

Section: Introductionmentioning

confidence: 99%

Delayed spontaneous remission of acquired factor V inhibitor refractory to immunosuppressive therapy with pregnancy-associated improvement

Ceglédi¹,

Dolgos²,

Fekete³

et al. 2023

Pathol. Oncol. Res.

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Introduction: Acquired factor V inhibitor (AFVI) is a rare autoimmune bleeding disorder. The treatment of AFVI is challenging, and patients often require both bleeding control and inhibitor eradication.Methods: We conducted a retrospective analysis of the medical records of a 35-year-old Caucasian woman who presented with severe AFVI-induced bleeding and subsequent immunosuppressive therapy.Results: To provide haemostasis, rFVIIa was given with good efficacy. The patient was treated with various combinations of immunosuppressive regimens over the course of 2.5 years, including plasmapheresis plus immunoglobulins, dexamethasone + rituximab, cyclophosphamide + dexamethasone + rituximab + cyclosporine, cyclosporin + sirolimus + cyclophosphamide + dexamethasone, bortezomib + sirolimus + methylprednisolone, and sirolimus + mycophenolate mofetil. Although these treatment modalities resulted in intermittent partial reversals of AFVI over 2.5 years, eventually the inhibitor became therapy-resistant. However, following the discontinuation of all immunosuppressive therapy, the patient experienced a partial spontaneous remission, which was followed by a pregnancy. During the pregnancy, the FV activity increased to 54% and the coagulation parameters returned to normal levels. The patient underwent Caesarean section without any bleeding complications and delivered a healthy child.Discussion: The use of an activated bypassing agent for bleeding control is effective in patients with severe AFVI. The presented case is unique because the treatment regimens included multiple combinations of immunosuppressive agents. This demonstrates that AFVI patients may undergo spontaneous remission even after multiple courses of ineffective immunosuppressive protocols. Additionally, pregnancy-associated improvement of AFVI is an important finding that warrants further investigation.

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Acquired Factor V Inhibitor after Coronavirus Disease 2019 (COVID-19)

Cited by 5 publications

References 16 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

Autoimmune Diseases Affecting Hemostasis: A Narrative Review

Delayed spontaneous remission of acquired factor V inhibitor refractory to immunosuppressive therapy with pregnancy-associated improvement

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