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Case report
Diagnostic delay in a case of T-cell neurolymphomatosis
  1. Vivien Li1,
  2. Zane Jaunmuktane1,2,
  3. Kate Cwynarski3 and
  4. Aisling Carr4
  1. 1National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London, UK
  2. 2Department of Clinical and Movement Neurosciences, University College London, London, UK
  3. 3Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK
  4. 4Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London, UK
  1. Correspondence to Dr Aisling Carr; aisling.carr{at}nhs.net

Abstract

A 69-year-old woman presented with severe subacute painful meningoradiculoneuritis. Neurophysiology showed a patchy, proximal axonal process with widespread denervation. Cerebrospinal fluid (CSF) was lymphocytic (normal T-cell predominant) with negative cytology. MRI revealed multiple sites of enhancement, but fluorodeoxyglucose positron emission tomography was negative. Bone marrow aspirate and trephine (BMAT) showed no evidence of a lymphoproliferative condition. Right brachial plexus biopsy demonstrated mixed T-cell/B-cell endoneurial inflammation not fulfilling criteria for vasculitis. She was stabilised with high-dose steroids and cyclophosphamide, followed by mycophenolate for inflammatory myeloradiculoneuritis. However, symptoms recurred when prednisolone was weaned. Although T-cell receptor gene analysis from the initial CSF demonstrated clonal rearrangements, it was only when the same clones were identified on two repeat BMATs and CSF that T-cell neurolymphomatosis, an exceedingly rare condition, was diagnosed. This case highlights the diagnostic challenge in peripheral neurolymphomatosis related to patchy disease, variable sensitivity and specificity of investigative tools, and the influence of therapies on traditional cytological definitions of lymphoma. The clinical picture, exhaustive exclusion of alternative causes and the persistence of an abnormal T-cell clone ultimately lead to a diagnostic consensus between specialist neurology and haematology clinicians.

  • neuromuscular disease
  • peripheral nerve disease
  • haematology (incl blood transfusion)
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Footnotes

  • Twitter @Afitz3

  • Contributors AC identified the case, had the idea for the article and is the guarantor. VL performed the literature search and wrote the article. ZJ contributed to the interpretation of the data and revised the manuscript critically for important intellectual content. AC and KC managed the case. All authors approved the final version.

  • 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 Next of kin consent obtained.

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

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