A rare case of acquired factor V inhibitor, during treatment with dabigatran for chronic atrial fibrillation, successfully treated with bypassing agents

Giuffrida, Gaetano; Markovic, Uroš; Nicolosi, Daniela; Calafiore, Valeria

doi:10.1002/ccr3.3586

Cited by 2 publications

(1 citation statement)

References 14 publications

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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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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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透析導入後に自己免疫性後天性凝固第ⅴ因子欠乏症を発症し，脳出血を呈した血液透析患者の1例

Ishisaka

Endo

Tamai

et al. 2023

Nihon Toseki Igakkai Zasshi

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Keywords： hemodialysis, acquired factor V inhibitor, cerebral hemorrhage 〈Abstract〉 In X 3 years, a woman in her 70s was started on hemodialysis. Her PT and APTT were 11 and 24 sec, respectively, at that time. Nafamostat mesylate had been used during dialysis as of October X 1, after she developed gastric antral vascular ectasia. She was treated with antimicrobial agents for fever and cough in February X, but did not improve. She was consequently admitted to our facility. Aside from pneumonia, abnormal coagulation was observed (PT： 54 sec, APTT： 215 sec). Coagulation factor V activity was significantly reduced, and factor V inhibitor and antiFV autoantibodies were detected in the blood, indicating the presence of acquired factor V inhibitor. The patient showed a right temporal lobe hemorrhage on the 33rd day of hospitalization, and 10 units of platelet concentrate were transfused. On the 34th day, oral prednisolone at 20 mg/day was initiated. On the

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A rare case of acquired factor V inhibitor, during treatment with dabigatran for chronic atrial fibrillation, successfully treated with bypassing agents

Abstract: Acquired factor V inhibitor represents a rare condition, described only in case reports. The use of activated bypassing agents in bleeding control could be of aid and improve the survival in symptomatic patients with acquired factor V inhibitor.

Cited by 2 publications

References 14 publications

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

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

透析導入後に自己免疫性後天性凝固第ⅴ因子欠乏症を発症し，脳出血を呈した血液透析患者の1例

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