Some patients with
thrombotic thrombocytopenic purpura (
TTP) are refractory to standard treatment regimens comprised of
plasma exchange (PEX) and
steroids. This report describes a 40-year-old woman with refractory
TTP who achieved complete remission (CR) in response to
rituximab. She was referred to our institution from a rural hospital with
purpura of the extremities, severe
thrombocytopenia,
anemia, and rapidly progressive disturbance of consciousness.
TTP was diagnosed based on the clinical symptoms of
TTP, low ADAMTS13 activity (<0.5%), and high ADAMTS13 inhibitor (4.4 BU/ml) titers. High-dose
prednisolone was immediately administered and PEX was started. This approach was initially effective, but the
thrombocytopenia and disturbance of consciousness worsened on the sixth day of treatment. We considered this patient to have refractory
TTP and administered weekly
rituximab. CR was achieved on day 20, and the disease status of this patient has remained stable over the long term. Our experience with this patient and five others who were similarly treated at our hospital over the past eight years indicates that
rituximab is effective for refractory
TTP.