Successful immunoadsorption of life-threatening bleeding in factor VIII inhibitor disease, but no long-term remission with anti-CD20 treatment

Abstract

A 62-year-old man and a 64-year-old woman presented to our institution with acquired haemophilia A. They both developed life-threatening bleeding. Immunoadsorption using protein A columns was used to rapidly lower factor VIII inhibitor levels. Immunosuppression with steroids and the anti-CD20 antibody, rituximab, was instituted. Yet their effects were either partial or complicated by an early relapse. Repetitive cyclophosphamide administration led to a sustained immunological response. While immunoadsorption appears effective and safe to lower factor VIII inhibitor levels, it seems that further preferably randomised controlled trials are needed to establish the value of rituximab versus the standard immunosuppressive regime comprising cyclophosphamide.

Figure 1

Decisive laboratory parameters and treatments for patient A and patient B. The symbols in the figure mark the following events: ▪ course of PTT values in s; ▴ course of inhibitor level in Bethesda Units (BU)/mL; ○ course of factor VIII level in % of normal; administration of rituximab; immunoadsorption treatment.