Challenges in management of unusual acquired factor V deficiency

Abstract

Rationale:Acquired inhibitors of coagulation are antibodies that either inhibit the activity or increase the clearance of a clotting factor. Acquired factor V deficiency is a rare coagulation disorder, and it can sometimes be life threatening.Patient concerns:We describe a case of a 90-year-old Japanese male with acquired factor V deficiency. He was previously misdiagnosed with congenital factor V deficiency when he presented with hemoptysis and a negative factor V inhibitor test result at a different hospital 5 years earlier. Coagulopathy recurred with ecchymosis when he sustained a bruise after falling on a bush.Diagnosis:Although the factor V inhibitor test result was negative and a mixing study suggested a deficiency pattern, we diagnosed the patient with acquired factor V deficiency on the basis of no history of bleeding diathesis, a lack of response to multiple fresh frozen plasma transfusion, and clinical response to corticosteroid therapy.Interventions:Intravenous methylprednisolone was administered at 500 mg/day for 3 days, followed by oral prednisolone at 1 mg/kg/day.Outcomes:Coagulation test results improved and symptoms resolved 2 weeks after corticosteroid administration.Lessons:This case report suggests that clearance-facilitating antibodies exist without the presence of neutralizing inhibitors. When patients present with coagulation factor V deficiency in the absence of coagulation inhibitors, acquired factor V deficiency should also be considered.

Parahaemophilia: A Rare Case Report

“…In acute cases of severe bleeding, the addition of platelet concentrates may be helpful.Prognosis is good with early diagnosis and adequate treatment. 8,13 Conclusion:…”

Challenges in diagnosis and management of acquired factor V inhibitors

“…2 Immunosuppressive agents like corticosteroids with or without cyclophosphamide and rituximab have been successful in 80% of cases. 10,11 The remission rates were maximum in idiopathic cases and least with the worst prognosis in autoimmune disorders and malignancies. 2…”

Acquired Factor V Deficiency After Carbapenem Administration: A Case Report

“…Additionally, FV is associated with anticoagulation through the inactivation of factor VIII which is mediated by activated protein C [1,2]. FV is synthesized in the liver, and approximately 80% of the total content of FV is present in plasma, while the remaining 20% is retained in platelet alpha-granules [3,4].…”

“…Acquired FV deficiency was first described in 1955. The overall incidence of FV deficiency is approximately 1 case per 1 million [2,3]. The incidence of acquired FV deficiency is estimated to be 0.09-0.29 per 1 million [6].…”

“…The incidence of acquired FV deficiency is estimated to be 0.09-0.29 per 1 million [6]. The acquired form generally results from the production of antibodies against FV following the administration of fresh frozen plasma (FFP) or antibiotics, autoimmune diseases, malignancies, or idiopathic formation of FV inhibitors [1,3]. Similar to congenital FV deficiency, prothrombin time (PT) and activated partial thromboplastin time (aPTT) are prolonged in acquired FV deficiency, where FV is at a low level.…”