Multimodality therapy of an acquired factor V inhibitor

Abstract

Acquired inhibitors of factor V are rare causes of clinlcai bleeding, whose severity ranges from mild to iife-threatening. Optimal treatment of patients with factor V inhibitors is uncertain. We report on our successful treatment approach in a patient with spontaneous, life-threatening intracraniai bleeding caused by a factor V Inhibitor. The patient deteriorated after Initial treatment with fresh-frozen plasma and platelet transfusions. He was subsequently treated with a combination of plasma exchange and chemotherapy, which led to complete recovery. Our experience suggests that plasma exchange may be iifesaving in cases of severe bleedlng caused by factor V inhibitors. The use of plasmapheresis in conjunction wlth chemotherapy Is an efflcacious and well-tolerated treatment and should be considered in patients with factor V inhibitors.

Treatment of acquired factor X inhibitor by plasma exchange with concomitant intravenous immunoglobulin and corticosteroids

“…Hence its use in patients with undetectable levels of factor X would be predicted to be ineffective as suggested by Mittal and Watson [14] and demonstrated in our case. Plasma exchange has been used for treatment of other inhibitors, including acquired inhibitors to factors VIII [15] and V [16,17]. There have only been one confirmed and one possible case of a factor X inhibitor where plasma exchange was utilized, with intravenous immunoglobulin and corticosteroids as adjuncts [4].…”

Acquired factor V inhibitors: a systematic review

“…The bypassing agent recombinant activated factor VII (rFVIIa) has also been successfully used in four cases with severe haemorrhage [83-85, 89, 90]. Plasmapheresis and immunoadsorption have been found to rapidly reduce antibody titers, thus making more efficacious the antihaemorrhagic and immunosuppressive regimens [56,59,63,66]. As such, a multimodal therapeutic approach encompassing a combination of replacement therapy, plasmapheresis and chemotherapy seems to be particularly effective in patients with severe haemorrhages [59].…”

“…Plasmapheresis and immunoadsorption have been found to rapidly reduce antibody titers, thus making more efficacious the antihaemorrhagic and immunosuppressive regimens [56,59,63,66]. As such, a multimodal therapeutic approach encompassing a combination of replacement therapy, plasmapheresis and chemotherapy seems to be particularly effective in patients with severe haemorrhages [59]. Moreover, high dose intravenous immunoglobulin (IVIG) has also been associated with a rapidly increasing FV activity [52,73], although the results of other studies were conflicting [67] (an anti-idiotypic effect or the saturation of the FcRn receptor has been suggested as possible mechanisms of action of IVIG) [91].…”

Persistant Hemothorax after Video-Assisted Thoracoscopic Surgery Secondary to Factor V Deficiency

“…The therapeutic strategies are focused on a) Control of the hemorrhage with the administration of fresh frozen plasma, platelet transfusions, prothrombin complex concentrates and recombinant Factor VII and, b) Eradication of antibodies, which has become the gold standard, with the use of systemic steroids, cyclophosphamide, rituximab, IV immunoglobulin, plasmapheresis and immune-absorption [11][12][13][14][15][16][17][18]. In this actual case, the patient received tranexamic acid, which is an antifibrinolysis complex with a potent inhibitory effect on the activation of fibrinolysis and, with higher concentrations, a noncompetitive inhibitor of plasmin.…”