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/m 2 /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.
CITATION STYLE
El Omri, H., Taha, R. Y., Gamil, A., Ibrahim, F., Al Sabah, H., Mahmoud, Z. O., … Yassin, M. A. (2015). Efficacy and safety of rituximab for refractory and relapsing thrombotic thrombocytopenic purpura: A cohort of 10 cases. Clinical Medicine Insights: Blood Disorders, 8, 1–7. https://doi.org/10.4137/CMBd.s25326
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