eprintid: 10092419
rev_number: 27
eprint_status: archive
userid: 608
dir: disk0/10/09/24/19
datestamp: 2020-02-28 11:06:14
lastmod: 2022-01-16 01:07:47
status_changed: 2020-02-28 11:06:14
type: article
metadata_visibility: show
creators_name: Li, V
creators_name: Jaunmuktane, Z
creators_name: Cwynarski, K
creators_name: Carr, A
title: Diagnostic delay in a case of T-cell neurolymphomatosis
ispublished: pub
divisions: UCL
divisions: B02
divisions: C07
divisions: D07
divisions: F84
note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
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.
date: 2019-12
date_type: published
official_url: https://doi.org/10.1136/bcr-2019-232538
oa_status: green
full_text_type: other
language: eng
primo: open
primo_central: open_green
verified: verified_manual
elements_id: 1737238
doi: 10.1136/bcr-2019-232538
pii: 12/12/e232538
lyricists_name: Jaunmuktane, Zane
lyricists_id: ZJAUN70
actors_name: Allington-Smith, Dominic
actors_id: DAALL44
actors_role: owner
full_text_status: public
publication: BMJ Case Reports
volume: 12
number: 12
article_number: e232538
event_location: England
citation:        Li, V;    Jaunmuktane, Z;    Cwynarski, K;    Carr, A;      (2019)    Diagnostic delay in a case of T-cell neurolymphomatosis.                   BMJ Case Reports , 12  (12)    , Article e232538.  10.1136/bcr-2019-232538 <https://doi.org/10.1136/bcr-2019-232538>.       Green open access   
 
document_url: https://discovery.ucl.ac.uk/id/eprint/10092419/13/Jaunmuktane_Diagnostic%20delay%20in%20a%20case%20of%20T-cell%20neurolymphomatosis_combined.pdf