Comparison of the Long-Term Remission of Rituximab and Conventional Treatment for Acquired Thrombotic Thrombocytopenic Purpura: A Systematic Review and Meta-Analysis

Abstract

The current systematic review and meta-analysis aimed to summarize the results of all available studies to compare the efficacies of rituximab and conventional treatment for acquired thrombotic thrombocytopenic purpura (TTP). Three investigators independently searched studies in the MEDLINE and EMBASE databases published before December 11, 2018. To be included in the meta-analysis, studies needed to be randomized-controlled or cohort studies comparing the efficacies of rituximab and conventional therapy for TTP treatment. The effect estimates and 95% confidence intervals (CIs) from each study were collected, and Mantel-Haenszel methods were used to pool the data. A total of 570 patients from 9 eligible studies were included in the meta-analysis (280 patients in the rituximab arm and 290 in the conventional treatment arm). Patients receiving rituximab in an acute phase to induce disease remission had a significantly lower relapse rate than those given conventional treatment (odds ratio [OR]: 0.40, 95% CI: 0.19-0.85, P = .02, I 2 = 43%). Similarly, the relapse rate in the rituximab group for preemptive therapy to prevent clinical relapse was also significantly lower than in the control group (OR: 0.09, 95% CI: 0.04-0.24, P < .00001, I 2 = 11%). Furthermore, the conventional treatment group had a significantly higher mortality rate than the rituximab group during the follow-up (OR: 0.41, 95% CI: 0.18-0.91, P = .03, I 2 = 0%). Rituximab offered high efficacy for the prevention of relapses and lower mortality rate in cases of acquired TTP.

Treatment of acquired thrombotic thrombocytopenic purpura without plasma exchange in selected patients under caplacizumab

“…The causal therapy of aTTP, however, is to eliminate potential triggers and initiate immunosuppressive therapy to treat the underlying autoimmune process. Steroids are widely used but rituximab, albeit off‐label in this indication, has demonstrated a significant reduction in relapse rates and mortality 11 . Caplacizumab therapy should be continued until ADAMTS13 activity has recovered to >10% in two consecutive measurements, indicating resolved autoantibody production.…”

Rituximab Twice Weekly for Refractory Thrombotic Thrombocytopenic Purpura in a Critically Ill Patient with Acute Respiratory Distress Syndrome

“…Furthermore, the conventional treatment group had a significantly higher mortality rate than the rituximab group during the follow-up. Rituximab offered high efficacy for the prevention of relapses and lower mortality rate in cases of acquired TTP [8]. According to our knowledge, rituximab twice weekly has never been used before.…”

Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

“…Additionally, this trial and many observational cohort studies suggest that rituximab given in the acute phase results in fewer relapses [ 20 , 154 , 155 , 156 , 157 ]. While a lower relapse rate did not reach statistical significance in all studies [ 158 , 159 ], a recent meta-analysis shows that rituximab administered during an acute iTTP episode not only lowers the relapse rate vs. control, but also reduces mortality [ 160 ]. Rituximab also appears to be effective in patients with refractory TTP or poor response to TPE [ 20 , 157 , 158 ].…”

“…In asymptomatic patients with ADAMTS13 activity persistently <10%, preemptive therapy with rituximab can effectively prevent relapse [ 144 , 160 , 198 ]. Cyclosporine has also been used for prophylaxis [ 199 ] and can be an option for patients who do not respond to rituximab.…”