Central nervous system involvement in adult-onset relapsing hemophagocytic lymphohistiocytosis responsive to maintenance treatment with anakinra
| Autor: | Francisco Javier García-Gómez, Natalia Fouz-Rosón, Salvador García Morillo, Carmen Vargas Lebrón, Ana Domínguez-Mayoral, Eloy Rivas-Infante, Marco Montes Cano, Jose Antonio Pérez de León, Juan Diego Guerra Hiraldo, Nayua Alkadi Fernández, Alicia Rodríguez Fernández, Guillermo Navarro Mascarell |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male 0301 basic medicine medicine.drug_class Immunology Central nervous system CNS Involvement Lymphohistiocytosis Hemophagocytic Disease activity 03 medical and health sciences 0302 clinical medicine Recurrence medicine Humans Immunology and Allergy Hemophagocytic lymphohistiocytosis Anakinra business.industry Brain Receptors Interleukin-1 medicine.disease Receptor antagonist Interleukin 1 Receptor Antagonist Protein Spinal Nerves Treatment Outcome 030104 developmental biology medicine.anatomical_structure Neurology Neurology (clinical) business 030217 neurology & neurosurgery Follow-Up Studies medicine.drug Inflammatory disorder |
| Zdroj: | Journal of Neuroimmunology. 355:577552 |
| ISSN: | 0165-5728 |
| DOI: | 10.1016/j.jneuroim.2021.577552 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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