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Langerhans cell histiocytosis with refractory central nervous system involvement responsive to infliximab
  1. Gurjit Chohan1,
  2. Yael Barnett1,2,
  3. John Gibson3,4,
  4. Stephen W R Reddel1,5,
  5. Michael H Barnett1,6
  1. 1Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia
  2. 2Department of Radiology, Royal North Shore Hospital, St Leonards, New South Wales, Australia
  3. 3Institute of Haematology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  4. 4Bosch Institute, University of Sydney, Camperdown, New South Wales Australia
  5. 5Department of Neurology, Concord Hospital, Concord, New South Wales, Australia
  6. 6Department of Neurology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  1. Correspondence to Dr Michael H Barnett, Brain and Mind Research Institute, Level 4, 94 Mallett Street, Camperdown NSW 2050 Australia; mbarnett{at}mail.usyd.edu.au

https://doi.org/10.1136/jnnp-2011-300575

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    We report a case of Langerhans cell histiocytosis (LCH) with central nervous system (CNS) involvement refractory to conventional first- and second-line treatments, but responsive to tumour necrosis factor α (TNFα) inhibition and discuss implications for pathogenesis of the disease.

    Case history

    A 48-year-old man presented with a 14-month history of gradually progressive disequilibrium, imbalance, oscillopsia, dysarthria and cognitive impairment. Sixteen months prior to presentation to our clinic, he developed a hard lump over the right frontal bone and biopsy confirmed the diagnosis of LCH on the basis of typical cellular morphology and strongly positive staining with CD1a and S100. Initial assessment suggested that the disease was limited to the skull, but progress CT imaging 2 months later demonstrated a separate, asymptomatic right intraconal orbital lesion. At that time he developed, but did not initially report, mild persistent disequilibrium and oscillopsia. Therapy (see online supplementary data for details) with prednisolone, etoposide and vinblastine resulted in regression of both lesions, but relapsed systemic disease with biopsy-confirmed bone marrow infiltration and abdominal masses 6 months later necessitated salvage therapy with monthly intravenous methotrexate and cytarabine, truncated at 2 months by an idiosyncratic febrile reaction and a possible seizure. Investigations including CT imaging …

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    Footnotes

    • Funding GC is the recipient of the Merck-Serono Multiple Sclerosis Fellowship at the Brain and Mind Research Institute.

    • Competing interests None.

    • Patient consent Obtained.

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