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

J Neuroimmunol. 2021 Jun 15:355:577552. doi: 10.1016/j.jneuroim.2021.577552. Epub 2021 Apr 2.

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.

Publication types

  • Case Reports

MeSH terms

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

Substances

  • Interleukin 1 Receptor Antagonist Protein
  • Receptors, Interleukin-1