Progressive Multifocal Leukoencephalopathy in Primary Immunodeficiencies.
J Clin Immunol
; 39(1): 55-64, 2019 01.
Article
en En
| MEDLINE
| ID: mdl-30552536
ABSTRACT
PURPOSE:
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.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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Leucoencefalopatía Multifocal Progresiva
/
Síndromes de Inmunodeficiencia
Tipo de estudio:
Observational_studies
/
Risk_factors_studies
Límite:
Adolescent
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Adult
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
J Clin Immunol
Año:
2019
Tipo del documento:
Article
País de afiliación:
Francia