Etienne Crickx1,2, Elsa Poullot3,4, Guillaume Moulis5,6,7, Radjiv Goulabchand8, Claire Fieschi9, Lionel Galicier9, Véronique Meignin10, Paul Coppo11, Richard Delarue12, Olivier Casasnovas13, Damien Roos-Weil14, Laurence de Leval15, Marie Parrens16, Marc Michel1, Jehan Dupuis17, Fabien Le Bras17, Virginie Fataccioli3,4, Nadine Martin-Garcia3,4, Bertrand Godeau1, Corinne Haïoun17, Philippe Gaulard3,4, Matthieu Mahévas1,2,18. 1. Service de médecine interne, Hôpital Henri-Mondor, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Est, Créteil, France. 2. Institut Necker Enfants Malades, INSERM U1151/CNRS UMS 8253, Université Paris Descartes, Sorbonne Paris Cité, Paris Cedex, France. 3. Département de Pathologie, Hôpital Henri-Mondor (AP-HP), Créteil, France. 4. Faculté de Médecine, Université Paris-Est, Inserm U955, Créteil, France. 5. Service de médecine Interne, CHU de Toulouse, Toulouse, France. 6. UMR 1027 Inserm-Université de Toulouse, Toulouse, France. 7. CIC 1436, CHU de Toulouse, Toulouse, France. 8. Service de médecine interne, maladies multi-organiques, Hôpital Saint-Eloi, CHRU Montpellier, Montpellier, France. 9. Service d'immunologie clinique, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Université Paris Diderot, Sorbonne Paris Cité, Paris, France. 10. Service d'anatomopathologie, Hôpital Saint-Louis (AP-HP), Paris, France. 11. Service d'hématologie, Centre de référence des microangiopathies thrombotiques, Hôpital Saint-Antoine (AP-HP), Sorbonne Universités, Paris, France. 12. Service d'hématologie, Hôpital Necker (AP-HP), Paris, France. 13. Service d'hématologie, Centre Hospitalier Universitaire de Dijon, Dijon, France. 14. Sorbonne Universités, UPMC Université Paris 06, AP-HP, GRC-11, Groupe de recherche clinique sur les hémopathies lymphoïdes (GRECHY), Hôpital Pitié-Salpétrière (APHP), Paris, France. 15. Institut de Pathologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. 16. Département de Pathologie, Hôpital Pessac, Bordeaux, France. 17. Unité Hémopathies Lymphoïdes, Hôpital Henri-Mondor (AP-HP), Créteil, France. 18. Unité Inserm U955, équipe 2, Hôpital Henri-Mondor, Assistance publique-hôpitaux de Paris, Créteil, France.
Abstract
OBJECTIVE: Angioimmunoblastic T-cell lymphoma (AITL) is frequently associated with autoimmune cytopenia (AIC). Whether such patients have a particular phenotype and require particular management is unclear. METHOD: Angioimmunoblastic T-cell lymphoma patients from the multicentric database of the Lymphoma Study Association presenting with AIC during disease course were included and matched to AITL patients without AIC (1/5 ratio). RESULTS: At diagnosis, AIC patients (n = 28) had more spleen and bone marrow involvement (54% vs 19% and 71% vs 34%, P < 0.001), Epstein-Barr virus replication (89% vs 39%, P < 0.001), gamma globulin titers (median 23 vs 15 g/L, P = 0.002), and proliferating B cells and plasmablasts in biopsies, as compared to control patients (n = 136). The 28 AIC patients had 41 episodes of AIC, diagnosed concomitantly with AITL in 23 (82%) cases. After a median follow-up of 24 months (range 3-155), 10 patients relapsed, all associated with AITL relapse. CONCLUSION: Our results provide new insight into AIC associated with AITL by highlighting the significant interplay between AITL and B-cell activation leading to subsequent autoimmunity.
OBJECTIVE:Angioimmunoblastic T-cell lymphoma (AITL) is frequently associated with autoimmune cytopenia (AIC). Whether such patients have a particular phenotype and require particular management is unclear. METHOD:Angioimmunoblastic T-cell lymphomapatients from the multicentric database of the Lymphoma Study Association presenting with AIC during disease course were included and matched to AITL patients without AIC (1/5 ratio). RESULTS: At diagnosis, AICpatients (n = 28) had more spleen and bone marrow involvement (54% vs 19% and 71% vs 34%, P < 0.001), Epstein-Barr virus replication (89% vs 39%, P < 0.001), gamma globulin titers (median 23 vs 15 g/L, P = 0.002), and proliferating B cells and plasmablasts in biopsies, as compared to control patients (n = 136). The 28 AICpatients had 41 episodes of AIC, diagnosed concomitantly with AITL in 23 (82%) cases. After a median follow-up of 24 months (range 3-155), 10 patients relapsed, all associated with AITL relapse. CONCLUSION: Our results provide new insight into AIC associated with AITL by highlighting the significant interplay between AITL and B-cell activation leading to subsequent autoimmunity.
Authors: Sriman Swarup; Jonathan Kopel; Kyaw Zin Thein; Kaiser Tarafdar; Khatrina Swarup; Seshadri Thirumala; Donald P Quick Journal: Am J Med Sci Date: 2020-09-03 Impact factor: 3.462