Progressive Multifocal Leukoencephalopathy in Primary Immunodeficiencies

Autor: Hadjadj, Jérôme, Guffroy, Aurélien, Delavaud, Christophe, Taieb, Guillaume, Meyts, Isabelle, Fresard, Anne, Streichenberger, Nathalie, L’honneur, Anne-Sophie, Rozenberg, Flore, Aguilar, Claire, Rosain, Jérémie, Picard, Capucine, Mahlaoui, Nizar, Lecuit, Marc, Hermine, Olivier, Lortholary, Olivier, Suarez, Felipe, D'Aveni, Maud, Notarantonio, Anne, Bertrand, Allan, Boulangé, Laura, Pochon, Cécile, Rubio, Marie
Přispěvatelé: Université Paris Descartes - Faculté de Médecine (UPD5 Médecine), Université Paris Descartes - Paris 5 (UPD5), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Référence National des Maladies Auto-Immunes Systémique Rares, CHU Strasbourg, Département de neurologie [Montpellier], Hôpital Gui de Chauliac [Montpellier]-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université Montpellier 1 (UM1)-Université de Montpellier (UM), Catholic University of Leuven - Katholieke Universiteit Leuven (KU Leuven), University Hospitals Leuven [Leuven], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Institut NeuroMyoGène (INMG), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Virologie [CHU Cochin], Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre d'infectiologie Necker-Pasteur [CHU Necker], Institut Pasteur [Paris]-CHU Necker - Enfants Malades [AP-HP], Centre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Imagine - Institut des maladies génétiques (IMAGINE - U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service des Maladies infectieuses et tropicales [CHU Necker], Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier]-Université de Montpellier (UM), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris Descartes - Paris 5 (UPD5)-CHU Necker - Enfants Malades [AP-HP]-Institut des Maladies Génétiques Imagine [Paris], Institut des Maladies Génétiques Imagine [Paris], Ingénierie Moléculaire et Physiopathologie Articulaire (IMoPA), Université de Lorraine (UL)-Centre National de la Recherche Scientifique (CNRS), Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université de Montpellier (UM), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Institut Pasteur [Paris] (IP)-CHU Necker - Enfants Malades [AP-HP], Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)
Rok vydání: 2018
Předmět:
0301 basic medicine
Male
MESH: Immunotherapy
medicine.medical_treatment
viruses
[SDV]Life Sciences [q-bio]
T-Lymphocytes
Azathioprine
0302 clinical medicine
Demyelinating disease
Immunology and Allergy
ComputingMilieux_MISCELLANEOUS
B-Lymphocytes
MESH: Middle Aged
Progressive multifocal leukoencephalopathy
Leukoencephalopathy
Progressive Multifocal
virus diseases
Immunosuppression
Middle Aged
JC Virus
3. Good health
[SDV.IMM.IA]Life Sciences [q-bio]/Immunology/Adaptive immunology
MESH: Young Adult
combined immunodeficiencies
[SDV.IMM]Life Sciences [q-bio]/Immunology
Rituximab
Female
MESH: Rituximab
Immunotherapy
medicine.drug
primary immunodeficiencies
Adult
medicine.medical_specialty
MESH: Immunologic Deficiency Syndromes
Adolescent
Immunology
immunosuppressive therapy
MESH: JC Virus
polyomavirus JC
MESH: Lymphopenia
03 medical and health sciences
Combined immunodeficiencies
Young Adult
MESH: B-Lymphocytes
Lymphopenia
medicine
Humans
MESH: Azathioprine
Retrospective Studies
MESH: Adolescent
MESH: Humans
business.industry
Tumor Necrosis Factor-alpha
MESH: Leukoencephalopathy
Progressive Multifocal
Immunologic Deficiency Syndromes
Retrospective cohort study
MESH: Adult
MESH: Retrospective Studies
medicine.disease
Dermatology
MESH: Male
030104 developmental biology
MESH: T-Lymphocytes
MESH: Tumor Necrosis Factor-alpha
Complication
business
MESH: Female
030217 neurology & neurosurgery
[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Zdroj: Journal of Clinical Immunology
Journal of Clinical Immunology, Springer Verlag, 2019, 39 (1), pp.55-64. ⟨10.1007/s10875-018-0578-8⟩
Journal of Clinical Immunology, 2019, 39 (1), pp.55-64. ⟨10.1007/s10875-018-0578-8⟩
ISSN: 1573-2592
0271-9142
DOI: 10.1007/s10875-018-0578-8⟩
Popis: International audience; Purpose:Progressive multifocal leukoencephalopathy (PML) is a rare but severe demyelinating disease caused by the polyoma-virus JC (JCV) in immunocompromised patients. We report a series of patients with primary immune deficiencies (PIDs) who developed PML. Methods:Retrospective observational study including PID patients with PML. Clinical, immunological, imaging features, and outcome are provided for each patient. Results:Eleven unrelated patients with PIDs developed PML. PIDs were characterized by a wide range of syndromic or genetically defined defects, mostly with combined B and T cell impairment. Genetic diagnosis was made in 7 patients. Before the development of PML, 10 patients had recurrent infections, 7 had autoimmune and/or inflammatory manifestations, and 3 had a history of malignancies. Immunologic investigations showed CD4 + lymphopenia (median 265, range 50-344) in all cases. Six patients received immunosuppressive therapy in the year before PML onset, including prolonged steroid therapy in 3 cases, rituximab in 5 cases, anti-TNF-α therapy, and azathioprine in 1 case each. Despite various treatments, all but 1 patient died after a median of 8 months following PML diagnosis. Conclusion: PML is a rare but fatal complication of PIDs. Many cases are secondary to immunosuppressive therapy warranting careful evaluation before initiation subsequent immunosuppression during PIDs.
Databáze: OpenAIRE
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