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31 results on '"Grosleron S"'

1. Atteintes neurologiques au cours du syndrome VEXAS : série rétrospective multicentrique nationale de 30 patients

Author

Bert-Marcaz, C., primary, Fortanier, E., additional, Briantais, A., additional, Faucher, B., additional, Bourguiba, R., additional, Swiader, L., additional, Schleinitz, N., additional, Jean, R., additional, Bigot, A., additional, Marianetti-Guingel, P., additional, Kostine, M., additional, Outh, R., additional, Dieudonné, Y., additional, Lazaro, E., additional, Vial, G., additional, Palat, S., additional, Frachet, S., additional, De Almeida Chaves, S., additional, Vinzio, S., additional, Robert, M., additional, comont, T., additional, Dion, J., additional, Lacombe, V., additional, Langlois, V., additional, Jachiet, V., additional, Decker, P., additional, Grosleron, S., additional, Broner, J., additional, Guilpain, P., additional, Samson, M., additional, Terrier, B., additional, Georgin-Lavialle, S., additional, Mekinian, A., additional, Delmont, E., additional, and Ebbo, M., additional

Published
2023
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9. Bordetella bronchiseptica infection

Author

Ducours, M., primary, Rispal, P., additional, Danjean, M.P., additional, Imbert, Y., additional, Dupont, E., additional, Traissac, E.M., additional, and Grosleron, S., additional

Published
2017
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10. Brigatinib in patients with ALK‐positive anaplastic large cell lymphoma who have failed brentuximab vedotin.

Author

Veleanu, L., Tesson, B., Lamant, L., Marçais, A., Bruneau, J., Kaltenbach, S., Brouzes, C., Degoutte, C., Moraly, J., Villarese, P., Lhermitte, L., Latiri, M., Hure, G., Chauchet, A., Delette, C., Grosleron, S., Toussaint, E., Cabrera, Q., Brice, P., and Gros, F.

Subjects
ANAPLASTIC large-cell lymphoma, ANAPLASTIC thyroid cancer, T-cell lymphoma
Abstract

ALK-inhibitors have shown interesting results in relapsed ALK+ ALCL, but in these studies, most pts had not received prior BV, which does not correspond to current standards of treatment in adults. ALK-positive anaplastic large cell lymphoma (ALK+ ALCL) patients (pts) who have failed brentuximab vedotin (BV) have a poor prognosis with a median OS after BV failure of 2.9 months and 2-year OS of 27.1% (Chihara D, 2019). Brigatinib in patients with ALK-positive anaplastic large cell lymphoma who have failed brentuximab vedotin. [Extracted from the article]

Published
2023
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29. Neurological manifestations in patients with VEXAS syndrome.

Author

Bert-Marcaz C, Fortanier É, Briantais A, Faucher B, Bourguiba R, Swiader L, Schleinitz N, Corazza G, Jean R, Bigot A, Marianetti-Guingel P, Kostine M, Outh R, Dieudonné Y, Lazaro E, Vial G, Palat S, Frachet S, De Almeida Chaves S, Vinzio S, Sacré K, Robert M, Comont T, Dion J, Girardie P, Lacombe V, Langlois V, Jachiet V, Decker P, Moulinet T, Grosleron S, Broner J, Guilpain P, Samson M, Terrier B, Georgin-Lavialle S, Attarian S, Mekinian A, Delmont E, and Ebbo M

Subjects
Humans, Male, Retrospective Studies, Adult, Middle Aged, Female, Registries, France epidemiology, Young Adult, Aged, Adolescent, Nervous System Diseases etiology, Nervous System Diseases epidemiology, Mutation, Child, Ubiquitin-Activating Enzymes genetics
Abstract

Background: VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a recently described syndrome linked to somatic mutations in the UBA1 gene, causing systemic autoinflammatory manifestations. To date, few data are available concerning neurological manifestations. The aim of this study was to describe their prevalence, clinical spectrum and outcome under treatment., Methods: Retrospective multicentre study including patients with VEXAS syndrome from the French VEXAS Registry between November 2020 and March 2023. Additional cases were included after a national call for observations. Each patient with confirmed UBA1 somatic mutation and neurological manifestation was reviewed during multidisciplinary meetings. Clinical, radiological, biological characteristics, treatments, and outcome were described., Results: Of the 291 patients included in the French VEXAS Registry, 17 (6%) had central (CNS) or peripheral (PNS) neurological involvement, with 13 additional cases identified by the national call. Of the 30 patients included, 21 (70%) had PNS involvement and 9 (30%) CNS involvement. PNS involvements included polyneuropathy (n = 9), cranial nerve involvement (n = 7), non-length-dependent polyneuropathy (n = 5) and multiple mononeuropathy (n = 3). CNS involvements included encephalopathy (n = 6), lacunar cerebral infarcts (n = 4), posterior reversible encephalopathy syndrome (n = 3) and optic perineuritis (n = 2). Most neurological manifestations were improved by steroids (68%), steroid-sparing agents were used in 90% [most frequently ruxolitinib (n = 11), azacitidine (n = 8), tocilizumab (n = 4)], and mortality was 30% after a median follow-up of 4 years., Conclusions: Neurological manifestations may occur in a small but possibly underestimated proportion of patients with VEXAS syndrome, are heterogeneous and can involve both PNS and CNS., Competing Interests: Declarations. Conflicts of interest: No author has any conflict of interest to declare., (© 2025. Springer-Verlag GmbH Germany, part of Springer Nature.)

Published
2025
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30. Serious infections in patients with VEXAS syndrome: data from the French VEXAS registry.

Author

de Valence B, Delaune M, Nguyen Y, Jachiet V, Heiblig M, Jean A, Riescher Tuczkiewicz S, Henneton P, Guilpain P, Schleinitz N, Le Guenno G, Lobbes H, Lacombe V, Ardois S, Lazaro E, Langlois V, Outh R, Vinit J, Martellosio JP, Decker P, Moulinet T, Dieudonné Y, Bigot A, Terriou L, Vlakos A, de Maleprade B, Denis G, Broner J, Kostine M, Humbert S, Lifermann F, Samson M, Pechuzal S, Aouba A, Kosmider O, Dion J, Grosleron S, Bourguiba R, Terrier B, Georgin-Lavialle S, Fain O, Mekinian A, Morgand M, Comont T, and Hadjadj J

Subjects
Aged, Humans, Arthralgia, Azacitidine, Mutation, Retrospective Studies, Bacteriophages, Janus Kinase Inhibitors, Myelodysplastic Syndromes, Skin Diseases, Genetic
Abstract

Introduction: Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an acquired autoinflammatory monogenic disease with a poor prognosis whose determinants are not well understood. We aimed to describe serious infectious complications and their potential risk factors., Methods: Retrospective multicentre study including patients with VEXAS syndrome from the French VEXAS Registry. Episodes of serious infections were described, and their risk factors were analysed using multivariable Cox proportional hazards models., Results: Seventy-four patients with 133 serious infections were included. The most common sites of infection were lung (59%), skin (10%) and urinary tract (9%). Microbiological confirmation was obtained in 76%: 52% bacterial, 30% viral, 15% fungal and 3% mycobacterial. Among the pulmonary infections, the main pathogens were SARS-CoV- 2 (28%), Legionella pneumophila (21%) and Pneumocystis jirovecii (19%). Sixteen per cent of severe infections occurred without any immunosuppressive treatment and with a daily glucocorticoid dose ≤10 mg. In multivariate analysis, age >75 years (HR (95% CI) 1.81 (1.02 to 3.24)), p.Met41Val mutation (2.29 (1.10 to 5.10)) and arthralgia (2.14 (1.18 to 3.52)) were associated with the risk of serious infections. JAK inhibitors were most associated with serious infections (3.84 (1.89 to 7.81)) compared with biologics and azacitidine. After a median follow-up of 4.4 (2.5-7.7) years, 27 (36%) patients died, including 15 (56%) due to serious infections., Conclusion: VEXAS syndrome is associated with a high incidence of serious infections, especially in older patients carrying the p.Met41Val mutation and treated with JAK inhibitors. The high frequency of atypical infections, especially in patients without treatment, may indicate an intrinsic immunodeficiency., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Published
2024
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