Central nervous system involvement in adult-onset relapsing hemophagocytic lymphohistiocytosis responsive to maintenance treatment with anakinra

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...

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Veröffentlicht in:Journal of neuroimmunology 2021-06, Vol.355, p.577552-577552, Article 577552
Hauptverfasser: Hiraldo, Juan Diego Guerra, Domínguez-Mayoral, Ana, García-Gómez, Francisco Javier, Fouz-Rosón, Natalia, Rivas-Infante, Eloy, Cano, Marco Antonio Montes, Fernández, Alicia Rodríguez, Morillo, Salvador García, Fernández, Nayua Alkadi, de León, Jose Antonio Pérez, Mascarell, Guillermo Navarro, Lebrón, Carmen Vargas
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container_start_page 577552
container_title Journal of neuroimmunology
container_volume 355
creator Hiraldo, Juan Diego Guerra
Domínguez-Mayoral, Ana
García-Gómez, Francisco Javier
Fouz-Rosón, Natalia
Rivas-Infante, Eloy
Cano, Marco Antonio Montes
Fernández, Alicia Rodríguez
Morillo, Salvador García
Fernández, Nayua Alkadi
de León, Jose Antonio Pérez
Mascarell, Guillermo Navarro
Lebrón, Carmen Vargas
description 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. [Display omitted]
doi_str_mv 10.1016/j.jneuroim.2021.577552
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title Central nervous system involvement in adult-onset relapsing hemophagocytic lymphohistiocytosis responsive to maintenance treatment with anakinra
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