Central nervous system involvement in adult-onset relapsing hemophagocytic lymphohistiocytosis responsive to maintenance treatment with anakinra.
J Neuroimmunol
; 355: 577552, 2021 06 15.
Article
em En
| MEDLINE
| ID: mdl-33845282
ABSTRACT
A 43 year-old male presented with a relapsing and progressive systemic inflammatory disorder with central nervous system (CNS) involvement. After a two years follow up, he was diagnosed with hemophagocytic lymphohistiocytosis (HLH), based on clinical, laboratory and radiological findings. Treatment was started with anakinra, a recombinant humanised interleukin-1 (IL-1) receptor antagonist. Clinical response was good. Laboratory and radiological findings showed no disease activity throughout a five years follow-up period. Several immunosuppressive agents have been used in HLH without any good outcomes. This is the first case report of HLH with CNS involvement responsive to chronic treatment with anakinra.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Nervos Espinhais
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Encéfalo
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Linfo-Histiocitose Hemofagocítica
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Proteína Antagonista do Receptor de Interleucina 1
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adult
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Humans
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Male
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article