JOURNAL ARTICLE

Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura

Matthieu Jestin, Ygal Benhamou, An-Sofie Schelpe, Elien Roose, François Provôt, Lionel Galicier, Miguel Hié, Claire Presne, Pascale Poullin, Alain Wynckel, Samir Saheb, Christophe Deligny, Aude Servais, Stéphane Girault, Yahsou Delmas, Tarik Kanouni, Alexandre Lautrette, Dominique Chauveau, Christiane Mousson, Pierre Perez, Jean-Michel Halimi, Anne Charvet-Rumpler, Mohamed Hamidou, Pascal Cathébras, Karen Vanhoorelbeke, Agnès Veyradier, Paul Coppo
Blood 2018 November 15, 132 (20): 2143-2153
30201758
Preemptive rituximab infusions prevent relapses in immune thrombotic thrombocytopenic purpura (iTTP) by maintaining normal ADAMTS13 activity. However, the long-term outcome of these patients and the potential adverse events of this strategy need to be determined. We report the long-term outcome of 92 patients with iTTP in clinical remission who received preemptive rituximab after identification of severe ADAMTS13 deficiency (activity <10%) during the follow-up. Thirty-seven patients had >1 iTTP episode, and the median cumulative relapse incidence before preemptive rituximab was 0.33 episode per year (interquartile range [IQR], 0.23-0.66). After preemptive rituximab, the median cumulative relapse incidence in the whole population decreased to 0 episodes per year (IQR, 0-1.32; P < .001). After preemptive rituximab, ADAMTS13 activity recovery was sustained in 34 patients (37%) during a follow-up of 31.5 months (IQR, 18-65), and severe ADAMTS13 deficiency recurred in 45 patients (49%) after the initial improvement. ADAMTS13 activity usually improved with additional courses of preemptive rituximab. In 13 patients (14%), ADAMTS13 activity remained undetectable after the first rituximab course, but retreatment was efficient in 6 of 10 cases. In total, 14 patients (15%) clinically relapsed, and 19 patients (20.7%) experienced benign adverse effects. Preemptive rituximab treatment was associated with a change in ADAMTS13 conformation in respondent patients. Finally, in the group of 23 historical patients with iTTP and persistently undetectable ADAMTS13 activity, 74% clinically relapsed after a 7-year follow-up (IQR, 5-11). In conclusion, persistently undetectable ADAMTS13 activity in iTTP during remission is associated with a higher relapse rate. Preemptive rituximab reduces clinical relapses by maintaining a detectable ADAMTS13 activity with an advantageous risk-benefit balance.

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