Successful treatment of plasma exchange-refractory thrombotic thrombocytopenic purpura with rituximab: A case report.

ABSTRACT

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP), a subtype of thrombotic microangiopathy, has a very high fatality rate if there is no timely diagnosis or treatment. Here, we report a case of TTP refractory to high displacement plasma exchange, which was later successfully treated with rituximab. CASE SUMMARY: Here we report a case of refractory TTP in a 63-year-old woman with a low platelet count and decreased ADAMTS13 activity. Her platelet count was 9 × 109/L, hemoglobin level was 81 g/L, and ADAMTS13 was < 5%. She was diagnosed with thrombotic thrombocytopenic purpura. After 8 d of daily plasma exchange (PEX), her platelet levels were still low. However, after 6 d of treatment with rituximab, her platelet count increased and ADAMTS13 activity returned to normal. CONCLUSION: PEX can cure most patients, but the relapse rate can be up to 50%-60%. This case suggested that rituximab can improve the curative efficiency of PEX and prevent disease relapse in TTP.