Rapid elimination of a high-titer spontaneous factor V antibody by extracorporeal antibody-based immunoadsorption and immunosuppression

Abstract

We report on the rapid elimination of a potent spontaneous factor V antibody of undetermined etiology by extracorporeal immunoadsorption on sepharose-bound polyclonal sheep antibodies to human immunoglobulins (Ig-Therasorb, Baxter) in combination with immunosuppressive treatment. A 68-year-old woman presented with severe hematuria. Severe factor V deficiency (< 1%) caused by an antibody to factor V (26 BU/ml) was found. Extracorporeal immunoadsorption (8.245 +/- 553 ml plasma processed per session) led to an average reduction of the antibody titer by 75% per session. The procedure was well tolerated without any side effects. Hematuria ceased after three immunoadsorptions and complete elimination of the antibody was achieved after seven sessions (day 15), followed by a rapid increase of the factor V activity to normal levels. Treatment with cyclophosphamide and prednisone was started on day 6 and continued for 2 months. The patient remains in remission at 6 months. Extracorporeal immunoadsorption is a highly effective method for eliminating antibodies to factor V (or other clotting factors) in selected cases, i.e., in patients with severe bleeding tendency, high antibody titer, and low probability of a rapid spontaneous remission.

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].…”

Immunoadsorption as a rescue therapy in systemic lupus erythematosus: considerations on safety and efficacy

“…In addition, the plasma volume processed is not limited, even when patients are maintained on daily IAS [8,9], and IAS does not lead to increased antibody re-synthesis [10]. Unfortunately, IAS has never been prospectively tried against a control group not receiving adsorption therapy.…”

Desenvolvimento de inibidores do fator VIII na hemofilia A

“…Alguns grupos têm relatado o tratamento de pacientes com altos títulos de inibidor (anti-fator V e anti-FVIII) utilizando métodos extracorpóreos de retirada de anticorpos. [52][53][54][55] Entretanto, as metodologias de retirada extracorpórea dos inibidores do FVIII não são específicas para estas imunoglobulinas e promovem a retirada de moléculas de anticorpo da circulação importantes para outras respostas imunes. Adicionalmente, em alguns casos, o indivíduo pode perder proteínas plasmáticas essenciais, como a albumina.…”