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Haemophagocytic lymphohistiocytosis restricted to the central nervous system
  1. Senthil Velan Bhoopalan,
  2. Patrick K Campbell,
  3. Asim K Bag,
  4. Mihaela Onciu,
  5. Ashok Srinivasan
  1. St. Jude Children's Research Hospital, Memphis, TN, United States
  1. Correspondence to Dr Senthil Velan Bhoopalan, Oncology, Saint Jude Children's Research Hospital, Memphis, TN 38018, USA; senthil.bhoopalan{at}stjude.org

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An 8-year-old boy presented with fatigue, weakness and altered mental status necessitating intubation. Contrast-enhanced magnetic resonance imaging (MRI) of the brain revealed multifocal perivascular enhancement at the supratentorial juxtacortical white matter (figure 1A) and diffuse cerebellar leptomeningeal enhancement (figure 1B), suggestive of central nervous system (CNS) vasculitis versus infection versus acute disseminated encephalomyelitis (ADEM). Workup for infectious and autoimmune encephalitis was unremarkable. Methylprednisolone (20 mg/kg/day) was initiated for presumed ADEM. Brain biopsy, obtained due to continued deterioration in mental status, revealed extensive meningoencephalitis (figure 2A) and …

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Footnotes

  • Contributors SVB wrote the manuscript, with critical inputs from PKC, AKB and MO. AS supervised the work.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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