Ethnicity affects relapse-free survival in immune-mediated thrombotic thrombocytopenic purpura
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is caused by a severe, antibody-mediated deficiency of ADAMTS13 (A Disintegrin And Metalloproteinase with ThromboSpondin-1 motifs, 13th member) activity. The B-cell depleting agent rituximab is effective in restoring ADAMTS13 activity and therefore preventing relapses. However, the risk of relapse appears heterogeneous among patients, although the underlying causes are elusive. Preliminary reports suggested that African ancestry could be associated with decreased relapse-free survival (RFS). Data from the registry of the French National Thrombotic Microangiopathy Reference Center were used to further address the role of ethnicity on response and RFS after rituximab administration in the acute as well as in the preemptive setting. A total of 790 patients (134 patients of African ancestry and 656 patients of European ancestry) were included in the study. Time from rituximab administration to ADAMTS13 recovery was comparable between the two cohorts. Patients of African ancestry had inferior 3-year combined RFS after the first rituximab-treated episode compared to patients of European ancestry (p.
Authors
- V. Rieu (ORCID: https://orcid.org/0000-0003-4749-6907)
- Júlia Weisinger (ORCID: https://orcid.org/0009-0003-8337-2925)
- Maximilien Grall
- Raïda Bouzid (ORCID: https://orcid.org/0000-0003-1419-3758)
- Adrien Picod (ORCID: https://orcid.org/0000-0002-0639-5598)
- C. Cartery (ORCID: https://orcid.org/0000-0002-1343-9536)
- Aude Servais (ORCID: https://orcid.org/0000-0002-8165-8212)
- Laurent Gilardin (ORCID: https://orcid.org/0000-0001-9212-0859)
- Gabriel Choukroun (ORCID: https://orcid.org/0000-0003-0772-5656)
- Y. Benhamou (ORCID: https://orcid.org/0000-0001-8890-7341)
- Yahsou Delmas (ORCID: https://orcid.org/0000-0002-5814-3122)
- Valérie Châtelet (ORCID: https://orcid.org/0000-0002-3107-9087)
- Patricia Zunic
- Nihal Martis (ORCID: https://orcid.org/0000-0002-8932-3169)
- Élie Azoulay (ORCID: https://orcid.org/0000-0002-8162-1508)
- Manon Marie
- Anne Rumpler
- Mathieu Legendre (ORCID: https://orcid.org/0000-0001-8289-3049)
- Andreea-Adela Icleanu (ORCID: https://orcid.org/0009-0002-0576-5363)
- François Provôt (ORCID: https://orcid.org/0000-0003-0218-9153)
- Pierre Perez (ORCID: https://orcid.org/0000-0002-1900-2562)
- Sara Rovira Puig (ORCID: https://orcid.org/0000-0001-5905-3607)
- Pascale Poullin (ORCID: https://orcid.org/0000-0002-2748-4354)
- Camille Lepart
- Arnaud Jaccard (ORCID: https://orcid.org/0000-0001-8065-3199)
- Jehane Fadlallah
- Agnès Veyradier
- Jean-Michel Halimi
- Christophe Deligny
- Rutuja Gupte
- Maria del Mar Tolos Garcia
- Florian Blanchard
- Benoit Suzon
- Alexandre Hertig
- Simon Ville
- David Ribes
- Timon Albrecht (ORCID: https://orcid.org/0009-0008-8314-1365)
- Bérangère Joly
- Paul Coppo
- Margot Dierickx
- Jean-Francois Augusto
- Daniela Greco
- Moglie Le Quintrec
- Thomas Papo
- Loïc Lièvre
Institutions
- Semmelweis University (HU)
- Délégation Paris 5 (FR)
- Hôpital Necker-Enfants Malades (FR)
- Royal College of Surgeons in Ireland (IE)
- University of Antwerp (BE)
- Inserm (FR)
- Université Fédérale de Toulouse Midi-Pyrénées (FR)
- Université Paris Cité (FR)
- University of Reunion Island (RE)
- Helmholtz-Zentrum Dresden-Rossendorf (DE)
- Centre Hospitalier Universitaire de Bordeaux (FR)
- Centre Hospitalier Universitaire de Nice (FR)
- Centre Hospitalier Universitaire de Nantes (FR)
- Sanquin (NL)
- Sorbonne Université (FR)
- Centre Hospitalier Universitaire de La Réunion (RE)
- Centre Hospitalier Universitaire de Besançon (FR)
- Hôpital Charles-Nicolle (FR)
- Université Sorbonne Paris Nord (FR)
- Centre de Recherche des Cordeliers (FR)
- Centre Hospitalier Universitaire de Tours (FR)
- Hôpital de la Conception (FR)
- Centre Hospitalier Universitaire Amiens-Picardie (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Hôpital Albert Calmette (FR)
- Centre Hospitalier Régional et Universitaire de Nancy (FR)
- Hospices Civils de Lyon (FR)
- Hôpital Saint-Antoine (FR)
- Centre Hospitalier Universitaire d'Angers (FR)
- Centre Hospitalier Universitaire de Caen Normandie (FR)
- CHU Dijon Bourgogne (FR)
- CIC Rennes (FR)
- Hôpital Charles-Nicolle (TN)
- Centre Hospitalier Universitaire de Limoges (FR)
- Pitié-Salpêtrière Hospital (FR)
- Hôpital Edouard Herriot (FR)
- Centre d'Investigation Clinique de Nantes (FR)
- Metabolism and Renal Physiology (FR)
- Centre Hospitalier Universitaire de Clermont-Ferrand (FR)
- Hôpital Lapeyronie (FR)
- Centre de Recherche Saint-Antoine (FR)
- Centre Hospitalier Universitaire de Martinique (MQ)
- Peuplements végétaux et bioagresseurs en milieu tropical (RE)
- Starlab Barcelona SLU (Spain) (ES)
- Hôpital Pasteur (FR)
- Hôpital Saint-Louis (FR)
- Hôpital Bichat-Claude-Bernard (FR)
- Centre Hospitalier Universitaire de Rouen (FR)
- Centre Hospitalier Universitaire de Rennes (FR)
- Centre Hospitalier de Valenciennes (FR)
- Hôpital Maison Blanche (FR)
- Hungarian Research Network (HU)
- Groupe de recherche clinique en anesthésie réanimation médecine périopératoire (FR)
- Université de Rouen Normandie (FR)
- Imation (United States) (US)
- Université de Caen Normandie (FR)
- KU Leuven (BE)
Publication Details
- Journal
- Haematologica
- Published
- 2026-01-08
- DOI
- https://doi.org/10.3324/haematol.2025.288789
- Primary Topic
- Complement system in diseases
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- European Commission
- Direction Générale de l’offre de Soins
- Research Executive Agency