Progressive Multifocal Leukoencephalopathy in Primary Immunodeficiencies.
Adolescent
Adult
Azathioprine
/ therapeutic use
B-Lymphocytes
/ immunology
Female
Humans
Immunologic Deficiency Syndromes
/ complications
Immunotherapy
/ methods
JC Virus
/ immunology
Leukoencephalopathy, Progressive Multifocal
/ etiology
Lymphopenia
/ immunology
Male
Middle Aged
Retrospective Studies
Rituximab
/ therapeutic use
T-Lymphocytes
/ immunology
Tumor Necrosis Factor-alpha
/ immunology
Young Adult
Progressive multifocal leukoencephalopathy
combined immunodeficiencies
immunosuppressive therapy
polyomavirus JC
primary immunodeficiencies
Journal
Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137
Informations de publication
Date de publication:
01 2019
01 2019
Historique:
received:
03
09
2018
accepted:
03
12
2018
pubmed:
16
12
2018
medline:
27
11
2019
entrez:
16
12
2018
Statut:
ppublish
Résumé
Progressive multifocal leukoencephalopathy (PML) is a rare but severe demyelinating disease caused by the polyomavirus JC (JCV) in immunocompromised patients. We report a series of patients with primary immune deficiencies (PIDs) who developed PML. Retrospective observational study including PID patients with PML. Clinical, immunological, imaging features, and outcome are provided for each patient. 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 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.
Identifiants
pubmed: 30552536
doi: 10.1007/s10875-018-0578-8
pii: 10.1007/s10875-018-0578-8
doi:
Substances chimiques
Tumor Necrosis Factor-alpha
0
Rituximab
4F4X42SYQ6
Azathioprine
MRK240IY2L
Types de publication
Journal Article
Observational Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
55-64Références
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