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Idiopathic Thrombotic Thrombocytopenic Purpura (idiopathic + thrombotic_thrombocytopenic_purpura)
Selected AbstractsRituximab as an adjunct to plasma exchange in TTP: A report of 12 cases and review of literature,JOURNAL OF CLINICAL APHERESIS, Issue 5 2008Sushama Jasti Abstract Idiopathic thrombotic thrombocytopenic purpura (TTP) is caused by the production of autoantibodies against the Von Willebrand factor cleaving enzyme. This provides a rationale for the use of rituximab in this disease. We report a retrospective review of 12 patients treated with rituximab for TTP refractory to plasma exchange. Eleven patients were treated during initial presentation, and one patient was treated for recurrent relapse. Ten patients responded to treatment. Median time to response after first dose of rituximab was 10 days (5,32). Of the 11 patients treated during initial presentation, nine remain free of relapse after a median follow-up of 57+ months (1+,79+). Two patients died during initial treatment. One patient was lost to follow-up 1 month after achieving complete response. The patient treated for recurrent disease during second relapse remained disease free for 2years, relapsed and was treated again with rituximab, and was in remission for 22 months. She relapsed again, was retreated, and has now been in remission for 21+ months. We conclude that rituximab is an useful addition to plasma exchange treatment in TTP, but its exact role and dosing need to be verified in prospective studies. J. Clin. Apheresis, 2008. © 2008 Wiley-Liss, Inc. [source] Sustained response with rituximab in patients with thrombotic thrombocytopenic purpura: A report of 13 cases and review of the literature,AMERICAN JOURNAL OF HEMATOLOGY, Issue 7 2009Huichung T. Ling Idiopathic thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease mediated by autoantibodies directed against ADAMTS-13. A number of small series and case reports have shown promising results with rituximab in refractory or relapsed TTP. In this report, we present 13 patients with TTP treated with rituximab. Twelve of the 13 patients (92%) achieved complete response; no subsequent relapses occurred with median follow-up of 24 months (range, 13,84 months). The addition of rituximab to standard therapy appears to be effective in sustaining long-term remission in TTP. However, the optimal dosing and timing of rituximab warrant further investigation. Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc. [source] Effect of blood group on idiopathic thrombotic thrombocytopenic purpuraJOURNAL OF CLINICAL APHERESIS, Issue 4 2009Lara Zuberi Abstract Thrombotic thrombocytopenic purpura (TTP) is a condition caused by deficiency of ADAMTS13 resulting in accumulation of ultra large Von Willebrand factor multimers (ULVWF), leading to micro thrombi in multiple organs. The varying susceptibilities of blood group antigens to ADAMTS13 have been demonstrated. A and B antigens are protective of VWF; and VWF purified from blood group O individuals has been shown to be cleaved faster by ADAMTS13 compared to VWF from blood group AB individuals. We proposed that there may be a difference in the incidence of blood groups in TTP patients compared with the general population. We felt this to be important for a life-threatening disease with poorly understood epidemiology. We report a retrospective analysis of 74 patients presenting from 1993 to 2008 with idiopathic TTP. We studied the incidence across various blood groups and also estimated the recurrence and mortality in each group. The incidence of various blood groups were as follows: O 36%, A 36%, B 25%, and AB 2%, compared with expected frequencies in the Detroit area: O 44%, A 33% B 20%, and AB 3%. There was a trend of lower than expected frequency of blood group O. There were 24 recurrences and 14 deaths, uniform across blood groups. We hypothesized that there may be an association between blood groups and the risk of TTP; however the differences in our study were not statistically significant. Recurrence and disease specific mortality did not appear to be impacted by blood group. J. Clin. Apheresis 2009. © 2009 Wiley-Liss, Inc. [source] Rituximab pharmacokinetics during the management of acute idiopathic thrombotic thrombocytopenic purpuraJOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 6 2010V. MCDONALD Summary.,Background:,Increasingly, patients with acute, idiopathic, antibody mediated thrombotic thrombocytopenic purpura (TTP) are being treated with rituximab to achieve a durable remission, however, there is the potential that it is removed by plasma exchange (PEX). Objectives:,To look at the pharmacokinetics and pharmacodynamics of rituximab in patients with acute idiopathic TTP undergoing PEX. Patients and methods:,Patients who received rituximab for acute idiopathic TTP (group 1, n = 30) and a control group (group 2, n = 3) of TTP patients in remission receiving rituximab electively as maintenance were included. Rituximab levels were measured before/after each infusion, before/after PEX and in follow-up. ADAMTS-13 activity, anti-ADAMTS-13 IgG and CD19% were measured to assess response. Results:,The median number of PEX to remission after rituximab was 10 (range 4,25). In group 1 there was no significant incremental rise in the peak serum rituximab level until dose 4. Trough levels were lower in patients who had had PEX since their last rituximab infusion. In the control group, there was an incremental rise in the peak serum rituximab level and all patients had detectable trough levels. The median fall in rituximab per PEX was 65%. All patients achieved CD19 < 1%. In group 1, the median time to undetectable rituximab was 5 months (range 0,12 months) and to B cell return was 7 months (range 3,24 months). ADAMTS-13 increased and anti-ADAMTS-13 fell after therapy. There were three deaths and two relapses in group 1. Relapse was not temporally related to B cell return. [source] | ||||||||||