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

• Authors: Andrea Ceglédi, National Institute of Hematology and Infectious Diseases, Saint Ladislaus Campus, Budapest, Hungary; János Dolgos, South Pest Central Hospital, National Institute of Hematology and Infectious Diseases; Mónika Fekete, Semmelweis University; László Gopcsa, National Institute of Hematology and Infectious Diseases, Saint Ladislaus Campus, Budapest, Hungary; Andrea Várkonyi, National Institute of Hematology and Infectious Diseases, Saint Ladislaus Campus, Budapest, Hungary; Beáta Vilimi, National Institute of Hematology and Infectious Diseases, Saint Ladislaus Campus, Budapest, Hungary; Gábor Mikala, National Institute of Hematology and Infectious Diseases, Saint Ladislaus Campus, Budapest, Hungary; Imre Bodó, Emory University
• Journal Title: PATHOLOGY & ONCOLOGY RESEARCH
• Volume: Volume 29
• Publisher: Springer Verlag | 2023-06-02
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.3389/pore.2023.1611250
• Subjects: Health Sciences, Public Health
• Keywords: factor V inhibitor; coagulopathy; gestation; bleeding disorder; autoimmune

Abstract

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.