{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T14:16:34Z","timestamp":1782828994033,"version":"3.54.5"},"reference-count":9,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T00:00:00Z","timestamp":1694736000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T00:00:00Z","timestamp":1694736000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Neurol"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>This is a unique case that describes the presentation, investigations, and disease trajectory of a fatal, clonal CD8-positive T-cell lymphoproliferative disorder in an otherwise healthy and immunocompetent patient with Epstein-Barr virus seronegative status. Central nervous system involving T-cell lymphoproliferative disorders are rare and typically encountered in the setting of immunocompromise. These disorders are often associated with aggressive cytomorphological features and characteristic magnetic resonance imaging patterns, which were not seen in this case.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>Here we describe a case of a 65\u00a0year-old male presenting with neuropsychiatric symptoms, truncal ataxia, and falls who\u2019s bone marrow, cerebrospinal fluid, and brain biopsy were consistent with a clonal CD8-positive T-cell lymphoproliferative disorder that did not meet existing World Health Organization criteria for classification as\u00a0T-cell lymphoma. The patient was treated with intrathecal methotrexate resulting in transient improvement of his symptoms followed by disease progression and death related to aspiration.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>This case highlights the importance of urgent and comprehensive work-up in patients with clinical features suggestive of lymphoma with central nervous system involvement, despite atypical imaging features and lack of cytomorphological features satisfying current World Health Organization classification criteria.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12883-023-03371-8","type":"journal-article","created":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T08:07:36Z","timestamp":1694765256000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["A unique case of a fulminant clonal CD8-positive T-cell lymphoproliferative disorder with CNS involvement"],"prefix":"10.1186","volume":"23","author":[{"given":"Lia","family":"Mesbah-Oskui","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jarrah","family":"Alabkal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Waleed","family":"Alduaij","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Priya S.","family":"Dhawan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,9,15]]},"reference":[{"issue":"7","key":"3371_CR1","doi-asserted-by":"publisher","first-page":"1720","DOI":"10.1038\/s41375-022-01620-2","volume":"36","author":"R Alaggio","year":"2022","unstructured":"Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022;36(7):1720\u201348.","journal-title":"Leukemia."},{"key":"3371_CR2","doi-asserted-by":"publisher","first-page":"4124","DOI":"10.1200\/JCO.2008.16.4558","volume":"26","author":"J Vose","year":"2008","unstructured":"Vose J, Armitage J, Weisenburger D. International peripheral T-cell and natural killer\/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol. 2008;26:4124\u201330.","journal-title":"J Clin Oncol"},{"issue":"10","key":"3371_CR3","doi-asserted-by":"publisher","first-page":"2233","DOI":"10.1200\/JCO.2005.07.109","volume":"23","author":"TN Shenkier","year":"2005","unstructured":"Shenkier TN, Blay JY, O\u2019Neill BP, et al. Primary CNS lymphoma of T-cell origin: a descriptive analysis from the international primary CNS lymphoma collaborative group. J Clin Oncol. 2005;23(10):2233\u20139.","journal-title":"J Clin Oncol"},{"issue":"12","key":"3371_CR4","doi-asserted-by":"publisher","first-page":"2257","DOI":"10.1038\/sj.leu.2403202","volume":"17","author":"JJ Van Dongen","year":"2003","unstructured":"Van Dongen JJ, Langerak AW, Bruggermann M, et al. Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT98-3936. Leukemia. 2003;17(12):2257\u2013317.","journal-title":"Leukemia"},{"issue":"2","key":"3371_CR5","doi-asserted-by":"publisher","first-page":"e65","DOI":"10.1016\/j.jinf.2005.10.013","volume":"53","author":"P Delobel","year":"2006","unstructured":"Delobel P, Godel A, Thebault S, et al. Transient clonal expansion of T-large granular lymphocytes during primary cytomegalovirus infection. J Infect. 2006;53(2):e65\u20137.","journal-title":"J Infect"},{"issue":"5","key":"3371_CR6","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1634\/theoncologist.2008-0036","volume":"13","author":"A Carbone","year":"2008","unstructured":"Carbone A, Gloghini A, Dotti G. EBV-Associated lymphoproliferative disorders: classification and treatment. Oncologist. 2008;13(5):577\u201385.","journal-title":"Oncologist"},{"issue":"3","key":"3371_CR7","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1212\/CPJ.0000000000000639","volume":"9","author":"M Khan","year":"2019","unstructured":"Khan M, Ney DE, Kleinshmidt-DeMasters BK, et al. CNS atypical T-cell lymphoproliferative disease following treatment with alemtuzumab. Neurol Clin Pract. 2019;9(3):273\u20136.","journal-title":"Neurol Clin Pract"},{"key":"3371_CR8","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1007\/s00401-007-0338-y","volume":"115","author":"MS Dulai","year":"2008","unstructured":"Dulai MS, Park CY, Howell WD, et al. CNS T-cell lymphoma: an under-recognized entity? Acta Neuropathol. 2008;115:345\u201356.","journal-title":"Acta Neuropathol"},{"issue":"3","key":"3371_CR9","doi-asserted-by":"publisher","first-page":"296","DOI":"10.1093\/neuonc\/noy192","volume":"21","author":"C Grommes","year":"2019","unstructured":"Grommes C, Robenstein JL, DeAngelis LM, et al. Comprehensive approach to diagnosis and treatment of newly diagnosed primary CNS lymphoma. Neuro Oncol. 2019;21(3):296\u2013305.","journal-title":"Neuro Oncol"}],"container-title":["BMC Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12883-023-03371-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12883-023-03371-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12883-023-03371-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,19]],"date-time":"2023-11-19T08:10:22Z","timestamp":1700381422000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcneurol.biomedcentral.com\/articles\/10.1186\/s12883-023-03371-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9,15]]},"references-count":9,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2023,12]]}},"alternative-id":["3371"],"URL":"https:\/\/doi.org\/10.1186\/s12883-023-03371-8","relation":{},"ISSN":["1471-2377"],"issn-type":[{"value":"1471-2377","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,9,15]]},"assertion":[{"value":"14 May 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 September 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 September 2023","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"The patient\u2019s surrogate has provided signed written informed consent for publication of descriptions or identifying materials in medical journals.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"329"}}