Rituximab to Abbreviate Plasma Exchange in Anti-CFH (Complement Factor H) Antibody Mediated Atypical HUS

Abstract

While terminal complement blockade with Eculizumab is the first line therapy for atypical HUS (Haemolytic uremic syndrome), lack of its availability and cost limits its use. Plasma exchange becomes a first line modality in the current scenario. However, exposure to large volumes of allogenic plasma and lack of skilled manpower are the limiting factors associated with it. Moreover, there is a subset of patients who fail to respond to plasma exchange.

Treatment and follow up records of the children with atypical HUS who did not respond to daily plasma exchange therapy and their course during follow up was reviewed.

Three children with positive anti-complement factor H antibody atypical HUS did not respond to daily plasma exchange and were  Rituximab administered after completing five daily treatments. It was seen that these children, initially non-responsive to plasma therapy, attained remission after Rituximab and did not require further plasma exchanges. The remission was sustained in long term with a follow up of 7 years, 4 years and 6 months respectively. Rituximab might be a useful alternative in inducing haematological remission in children with poor or no response to plasma therapy. This abbreviates the duration of plasma exchange, which not only avoids complications due to prolonged plasma therapy but also helps reducing the cost of therapy.