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Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center
Abstract
OBJECTIVE: To assess the efficacy and safety of rituximab in adults responding poorly to standard treatment for severe autoimmune thrombotic thrombocytopenic purpura. DESIGN: Open-label prospective study. Outcomes in the survivors were compared to those of 53 historical survivors who were given therapeutic plasma exchange alone or with vincristine. SETTING: Hospitals belonging to the Reference Network for Thrombotic Microangiopathies in France. PATIENTS: Twenty-two adults with either no response or a disease exacerbation when treated with intensive therapeutic plasma exchange. INTERVENTION: Add-on rituximab therapy, four infusions over 15 days. MEASUREMENTS AND MAIN RESULTS: One patient died despite two rituximab infusions. In the rituximab-treated patients, the time to a durable remission was significantly shortened (p = .03), although the plasma volume required to achieve a durable remission was not significantly different compared to the controls. Platelet count recovery occurred within 35 days in all 21 survivors, compared to only 78% of the historical controls (p < .02). Of the rituximab-treated patients, none had a relapse within the first year but three relapsed later on. In patients treated with rituximab, a rapid and profound peripheral B-cell depletion was produced, lasting for 9 months and correlating with higher a disintegrin and metalloproteinase with thrombospondin-13 activity and lower anti-a disintegrin and metalloproteinase with thrombospondin-13 antibody titers. These differences were no longer significant after 12 months. No severe side effects occurred. CONCLUSIONS: Adults with severe thrombocytopenic purpura who responded poorly to therapeutic plasma exchange and who were treated with rituximab had shorter overall treatment duration and reduced 1-yr relapses than historical controls.
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Citations
Cites
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura
Sadler, J. E. (2008) · Blood · doi:10.1182/blood-2008-02-078170 · Cited by 574
Rituximab: Expanding Role in Therapy for Lymphomas and Autoimmune Diseases
Rastetter, W., Molina, A., & White, C. A. (2004) · Annual Review of Medicine · doi:10.1146/annurev.med.55.091902.104249 · Cited by 213
Cited by
Thrombotic thrombocytopenic purpura
Joly, B. S., Coppo, P., & Veyradier, A. (2017) · Blood · doi:10.1182/blood-2016-10-709857 · Cited by 716
Self-citationConnelly‐Smith, L., Alquist, C. R., Aqui, N. A., Hofmann, J. C., Klingel, R., Onwuemene, O. A., Patriquin, C. J., Pham, H. P., Sanchez, A. P., Schneiderman, J., et al. (2023) · Journal of Clinical Apheresis · doi:10.1002/jca.22043 · Cited by 684
KDIGO 2024 Clinical Practice Guideline for the management of LUPUS NEPHRITIS
Rovin, B. H., Ayoub, I. M., Chan, T. M., Liu, Z.-H., Mejía-Vilet, J. M., & Floege, J. (2024) · Kidney International · doi:10.1016/j.kint.2023.09.002 · Cited by 517
The European Hematology Association Roadmap for European Hematology Research: a consensus document
Engert, A., Balduini, C., Brand, A., Coiffier, B., Cordonnier, C., Dohner, H., de Wit, T. D., Eichinger, S., Fibbe, W., Green, T., et al. (2016) · Haematologica · doi:10.3324/haematol.2015.136739 · Cited by 81
Metadata
- Authors
- Froissart, A., Buffet, M., Veyradier, A., Poullin, P., Provôt, F., Malot, S., Schwarzinger, M., Galicier, L., Vanhille, P., Vernant, J.-P., Bordessoule, D., Guidet, B., Azoulay, E., Mariotte, E., Rondeau, E., Mira, J.-P., Wynckel, A., Clabault, K., Choukroun, G., Presne, C., Pourrat, J., Hamidou, M., & Coppo, P.
- Venue
- Critical Care Medicine
- Published
- 2012-01
- Volume
- 40
- Issue
- 1
- Pages
- 104-111
- ISSN
- 0090-3493 (ISSN-L)
- OpenAlex
- W2028444448
- Cited by
- 291
- References
- 2
- FWCI
- 7.61
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. https://app.cochat.ai/papers/10.1097/ccm.0b013e31822e9d66
- The paper
- Froissart, A., Buffet, M., Veyradier, A., Poullin, P., Provôt, F., Malot, S., Schwarzinger, M., Galicier, L., Vanhille, P., Vernant, J.-P., Bordessoule, D., Guidet, B., Azoulay, E., Mariotte, E., Rondeau, E., Mira, J.-P., Wynckel, A., Clabault, K., Choukroun, G., . . . Coppo, P. (2012). Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Critical Care Medicine. https://doi.org/10.1097/ccm.0b013e31822e9d66
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.