{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T10:39:55Z","timestamp":1773830395820,"version":"3.50.1"},"reference-count":15,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"9","content-domain":{"domain":["ascopubs.org"],"crossmark-restriction":true},"short-container-title":["JCO Precis Oncol"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:sec>\n            <jats:title>PURPOSE<\/jats:title>\n            <jats:p>CNS involvement in North American adult T-cell leukemia\/lymphoma (NA-ATLL) remains poorly understood. This study examined the CNS involvement in patients with ATLL treated at a tertiary hospital in New York City.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>METHODS<\/jats:title>\n            <jats:p>CNS involvement was defined by positive cerebrospinal fluid (CSF) cytology, flow cytometry, positive CNS imaging, or neurological examination findings.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>RESULTS<\/jats:title>\n            <jats:p>\n              Among 94 patients with NA-ATLL, 21 (22.3%) had CNS involvement. CSF was involved in 13 patients at diagnosis and five at relapse. Magnetic resonance imaging detected brain and spinal involvement in 24% and 14% of the patients, respectively. Results of neurological examinations were abnormal in 33% and 14% of the patients at diagnosis and relapse, respectively. The\n              <jats:italic toggle=\"yes\">XPO1<\/jats:italic>\n              <jats:italic toggle=\"yes\">E571K<\/jats:italic>\n              mutation was found in two patients with extensive, treatment-refractory CNS disease, with a median overall survival (OS) of 2 months. Other mutations, including\n              <jats:italic toggle=\"yes\">KLF2<\/jats:italic>\n              and\n              <jats:italic toggle=\"yes\">PI3KCD<\/jats:italic>\n              , were noted in two patients. The median OS was 8.5 months, and the median relapse-free survival (RFS) was 6.5 months in our series. In most cases (5\/21), the lymphomatous phenotype appeared to have a direct mass-like extension, whereas in patients with predominant blood involvement tended to spread to the CSF by traversing the blood-brain barrier.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>CONCLUSION<\/jats:title>\n            <jats:p>\n              In this report, we describe the patterns of CNS involvement in ATLL and their association with mutations. We also describe two rapidly fatal cases with the\n              <jats:italic toggle=\"yes\">XPO1 E571K<\/jats:italic>\n              mutation, which may represent a novel therapeutic target for T-cell lymphomas.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1200\/po.23.00526","type":"journal-article","created":{"date-parts":[[2025,7,25]],"date-time":"2025-07-25T19:59:06Z","timestamp":1753473546000},"update-policy":"https:\/\/doi.org\/10.1200\/crossmark","source":"Crossref","is-referenced-by-count":1,"title":["CNS Involvement by North American-Adult T-cell Leukemia\/Lymphoma Is Associated With Discrete Dissemination Patterns and Molecular Profiles, Involving XPO1 E571 and KLF2\/PI3KCD in Selected Cases"],"prefix":"10.1200","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4723-6745","authenticated-orcid":true,"given":"Nishi","family":"Shah","sequence":"first","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"},{"name":"Winship Cancer Institute of Emory University, Atlanta, GA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melissa","family":"Suchanek","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"},{"name":"Rutgers, Robert Wood Johnson School of Medicine, Piscataway, NJ"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7778-0752","authenticated-orcid":true,"given":"Astha","family":"Thakkar","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4194-502X","authenticated-orcid":true,"given":"Riya Jayesh","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"},{"name":"Roswell Park, Comprehensive Cancer Center, Buffalo, NY"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4751-0852","authenticated-orcid":true,"given":"Shafia","family":"Rahman","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0180-2972","authenticated-orcid":true,"given":"Ana","family":"Acuna-Villaorduna","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"},{"name":"Department of Oncology, Icahn School of Medicine at Mount Sinai, New York, NY"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8419-1091","authenticated-orcid":true,"given":"Urvi","family":"Shah","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"},{"name":"Memorial Sloan Kettering Cancer Center, New York, NY"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0168-2165","authenticated-orcid":true,"given":"Diego","family":"Adrianzen Herrera","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY"},{"name":"University of Vermont Larner College of Medicine, Burlington, VT"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shira","family":"Slasky","sequence":"additional","affiliation":[{"name":"Department of Oncology, Montefiore Medical Center, Albert Einstein College of 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