Efficacy and Safety of Rituximab for Refractory and Relapsing Thrombotic Thrombocytopenic Purpura: A Cohort of 10 Cases

Halima El Omri ； Ruba Y. Taha ； Amna Gamil ； Firyal Ibrahim ； Hisham Al Sabah ； Zeinab O. Mahmoud ； Gianfranco Pittari ； Ibrahim Al HI jji ； Mohamed A. Yassin

《Clinical Medicine Insights: Blood Disorders》 2015期 (2015/12) Pp. 1-7

https://doi.org/10.4137/CMBD.S25326

並列摘要

OBJECTIVE: Idiopathic thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder mediated by autoantibodies directed against ADAMTS13. This provides a rationale for the use of rituximab in this disorder. We report our experience and the outcome of 10 cases of TTP (9 refractory and 1 relapsing) successfully treated with rituximab in combination with plasma exchange (PE) and other immunosuppressive treatments. METHODS: The diagnosis of TTP was based on clinical criteria and supported by severe deficiency of ADAMTS13 activity and presence of inhibitors in seven cases. Rituximab was started after a median of 18.6 sessions of PE (range: 5–35) at the dose of 375 mg/m2/week for 4–8 weeks. RESULTS: Complete remission was achieved in all patients after a median time of 14.4 days of the first dose (range: 6–30). After a median follow-up of 30 months (range: 8–78), eight patients were still in remission and two developed multiple relapses, treated again with the same therapy, and achieved complete responses; they are alive, and in complete remission after a follow-up of 12 and 16 months. CONCLUSION: Rituximab appears to be a safe and effective therapy for refractory and relapsing TTP. However, longer follow-up is recommended to assess relapse and detect possible long-term side effects of this therapy.

並列關鍵字

TTP ； plasma exchange ； refractory ； relapse ； rituximab