Protein A sepharose immunoadsorption: immunological and haemostatic effects in two cases of acquired haemophilia

Abstract

Summary. Acquired haemophilia is a life-threatening disorder caused by circulating auto-antibodies that inhibit factor VIII coagulant activity (FBIII:C). Immunoadsorption on protein A sepharose (IA-PA) was performed in two bleeding patients with acquired haemophilia: we observed a dramatic and quick decrease in the anti-FVIII:C inhibitor titre leading to a normal, albeit transient, haemostatic status. In one case, IA-PA was the only procedure which succeeded in stopping massive haemorrhage. In the second case, IA-PA reinforced the haemostatic effect of recombinant activated factor VII by increasing the endogenous plasma factor VIII level. The efficacy of IA-PA was sustained with immunosuppressive treatment introduced, respectively, 10 and 15 d before the IA-PA procedures. Our experience with IA-PA suggests that this extracorporeal anti-FVIII:C removal procedure is a valuable therapeutic tool for acquired haemophilia and can alleviate life-threatening haemorrhages.

Immunoadsorption with single‐use columns for the management of bleeding in acquired hemophilia A: A series of nine cases

“…Nevertheless it is common opinion that patients with acquired hemophilia suffering from a life threatening hemorrhage need quick control of hemostasis. A number of case reports have been published describing IA treatment of patients with either inherited or acquired hemophilia using different, mostly reusable adsorptioncolumns [12,19,20]. Data from the literature suggest that in acquired hemophilia, where inhibitors consist of autoantibodies, IA along with moderate immunosuppession is often successful in the complete elimination of the inhibitors with reported relapse rates of only 20% [4].…”

“…E-mail: brzoska@em.uni-frankfurt. de. episodes are reduced [12][13][14]. In contrast to inherited hemophilia eradication of the inhibitor in acquired hemophilia enables the patient to replenish the missing factor spontaneously, thus making IA a major treatment principle in this disease.…”

Efficacy of 2-chlorodeoxyadenosine in refractory factor VIII inhibitors in persons without hemophilia

“…5 Other agents used in the treatment of acquired inhibitors to factor VIII include cyclosporin, azathioprine, immunoadsorption, vincristine, 6-mercaptopurine, and intravenous immunoglobulin. [7][8][9] In the current investigation, 2-CDA was administered to 6 patients with inhibitors against factor VIII that failed to respond to other immunosuppressants. Although the exact mechanism of action of 2-CDA is not well characterized, its strong immunosuppressive properties are thought to be mediated through the Fas/Fas ligand pathway and subsequent activation of cellular apoptosis.…”

Acquired factor VIII inhibitors in oncohematology: A systematic review

“…Effective hemostasis can be achieved with correction of FVIII deficiency (FVIII concentrates, desmopressin), bypassing (activated prothrombin complex concentrates, recombinant factor VII activated), neutralizing (high dose immunoglobulin) or removal of the inhibitor (immunoadsorption or plasmapheresis) [26][27][28][29][30]. The choice between these strategies will depend on the site and severity of the bleed and patient characteristics.…”