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

Publisher: Elsevier/North-Holland Country of Publication: Netherlands NLM ID: 8109498 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1872-8421 (Electronic) Linking ISSN: 01655728 NLM ISO Abbreviation: J Neuroimmunol Subsets: MEDLINE

Original Publication: [Amsterdam] : Elsevier/North-Holland, c1981-

Brain/*diagnostic imaging ; Interleukin 1 Receptor Antagonist Protein/*administration & dosage ; Lymphohistiocytosis, Hemophagocytic/*diagnostic imaging ; Lymphohistiocytosis, Hemophagocytic/*drug therapy ; Spinal Nerves/*diagnostic imaging; Adult ; Brain/drug effects ; Brain/metabolism ; Follow-Up Studies ; Humans ; Interleukin 1 Receptor Antagonist Protein/metabolism ; Lymphohistiocytosis, Hemophagocytic/metabolism ; Male ; Receptors, Interleukin-1/agonists ; Receptors, Interleukin-1/metabolism ; Recurrence ; Spinal Nerves/drug effects ; Spinal Nerves/metabolism ; Treatment Outcome

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.
(Copyright © 2021 Elsevier B.V. All rights reserved.)

0 (Interleukin 1 Receptor Antagonist Protein)
0 (Receptors, Interleukin-1)

Date Created: 20210412 Date Completed: 20210908 Latest Revision: 20210908

20240829

10.1016/j.jneuroim.2021.577552

33845282