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

Abstract

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.

• Authors: Halima El Omri, Ruba Y Taha, Amna Gamil, Firyal Ibrahim, Hisham Al Sabah, Zeinab O Mahmoud, Gianfranco Pittari, Ibrahim Al HIjji, Mohamed A Yassin
• Journal: Clinical medicine insights. Blood disorders (Clin Med Insights Blood Disord) Vol. 8 Pg. 1-7 ( 2015) ISSN: 1179-545X [Print] United States
• PMID: 26052230 (Publication Type: Case Reports)