{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T10:13:52Z","timestamp":1788516832164,"version":"build-2803163510"},"reference-count":19,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T00:00:00Z","timestamp":1788480000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T00:00:00Z","timestamp":1788480000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>T\u2010cell prolymphocytic leukemia (T\u2010PLL) is a rare, aggressive mature T\u2010cell malignancy with limited therapeutic options. Alemtuzumab remains the backbone of therapy, and fit responders are consolidated with allogeneic stem cell transplantation (alloSCT). However, contemporary population\u2010level outcomes remain poorly defined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      All T\u2010PLL cases diagnosed between 2001 and 2023 were identified from the nationwide Netherlands Cancer Registry. Overall survival (OS) and survival after relapse (OS2) were analyzed in 220 patients. Detailed treatment\u2010line data were available for patients diagnosed from 2014 onward (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009120), enabling assessment of treatment sequencing, response, and progression\u2010free survival (PFS).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Median age at diagnosis was 71\u2009years; 56% were male. Overall, 54% received systemic therapy, of whom 25% underwent transplantation. Median OS was 13.5\u2009months; 2\u2010 and 5\u2010year OS were 36% and 10%, with no improvement over time. Alemtuzumab was the predominant frontline therapy and was associated with higher response and survival than other regimens. Consolidation with alloSCT provided the greatest likelihood of durable disease control, although only one\u2010third of treated patients were eligible. Survival after relapse remained poor (median OS2, 5\u20137\u2009months), and later treatment lines rarely produced durable benefit. Nearly half of patients were initially managed with observation; long\u2010term survival remained limited after progression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Alemtuzumab followed by alloSCT remains the most effective strategy for fit patients with T\u2010PLL, yet long\u2010term survival is uncommon and has not improved over two decades. High relapse rates and poor salvage outcomes underscore the need for novel targeted and immune\u2010based approaches and optimized post\u2010remission strategies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ejh.70312","type":"journal-article","created":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T09:33:33Z","timestamp":1788514413000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Treatment Patterns, Outcomes and Survival Trends in T\u2010Cell Prolymphocytic Leukemia: A Nationwide Population\u2010Based Study in the Netherlands"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6014-4810","authenticated-orcid":false,"given":"Mirian","family":"Brink","sequence":"first","affiliation":[{"name":"Department of Research and Development Netherlands Comprehensive Cancer Organisation (IKNL)  Utrecht the Netherlands"},{"name":"Department of Hematology University Medical Center Groningen, University of Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arie C.","family":"van der Spek","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Northwest Hospital  Alkmaar the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1014-0918","authenticated-orcid":false,"given":"Jeanette K.","family":"Doorduijn","sequence":"additional","affiliation":[{"name":"Department of Hematology Erasmus MC Cancer Institute, University Medical Center Rotterdam  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4655-6007","authenticated-orcid":false,"given":"Koen","family":"de Heer","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Flevoziekenhuis  Almere the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5205-361X","authenticated-orcid":false,"given":"Sabina","family":"Kersting","sequence":"additional","affiliation":[{"name":"Department of Hematology Hagaziekenhuis  The Hague the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0771-5231","authenticated-orcid":false,"given":"Lidwine W.","family":"Tick","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Maxima Medical Centre  Veldhoven the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doreen G.","family":"te Raa","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Hospital Gelderse Vallei  Ede the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanneke M.","family":"van der Straaten","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine St. Jansdal Hospital  Harderwijk the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-7733-6542","authenticated-orcid":false,"given":"Gerrit Jan","family":"Veldhuis","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Antonius Hospital  Sneek the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurens","family":"Laterveer","sequence":"additional","affiliation":[{"name":"Department of Hematology Isala Hospital  Zwolle the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rogier","family":"Mous","sequence":"additional","affiliation":[{"name":"Department of Hematology, UMC Utrecht Cancer Center University Medical Center Utrecht  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2139-3547","authenticated-orcid":false,"given":"Mark\u2010David","family":"Levin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Albert Schweitzer Hospital  Dordrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6430-0155","authenticated-orcid":false,"given":"Kees","family":"Meijer","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry University Medical Center Groningen, University of Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-0500-943X","authenticated-orcid":false,"given":"Andre","family":"Mulder","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry University Medical Center Groningen, University of Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2740-2873","authenticated-orcid":false,"given":"M.","family":"Nijland","sequence":"additional","affiliation":[{"name":"Department of Hematology University Medical Center Groningen, University of Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7120-898X","authenticated-orcid":false,"given":"Gerwin","family":"Huls","sequence":"additional","affiliation":[{"name":"Department of Hematology University Medical Center Groningen, University of Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3190-1891","authenticated-orcid":false,"given":"Arnon P.","family":"Kater","sequence":"additional","affiliation":[{"name":"Department of Hematology Amsterdam UMC, University of Amsterdam  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9864-5816","authenticated-orcid":false,"given":"Mar","family":"Bellido","sequence":"additional","affiliation":[{"name":"Department of Hematology University Medical Center Groningen, University of Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,4]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"crossref","first-page":"S239","DOI":"10.3816\/CLM.2009.s.018","article-title":"T\u2010Cell Prolymphocytic Leukemia","volume":"9","author":"Dearden C. 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A.","year":"2024","journal-title":"American Journal of Hematology"},{"key":"e_1_2_10_15_1","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1038\/s41408-025-01428-0","article-title":"Epidemiology and Survival of Patients With T\u2010Cell Prolymphocytic Leukemia","volume":"15","author":"Wolgast N.","year":"2025","journal-title":"Blood Cancer Journal"},{"key":"e_1_2_10_16_1","doi-asserted-by":"crossref","first-page":"6213","DOI":"10.1182\/blood-2023-184602","article-title":"Real\u2010World Evaluation of T\u2010Prolymphocytic Leukemia Outcomes Using the 2019 T\u2010PLL International Study Group Criteria","volume":"142","author":"Braunstein Z.","year":"2023","journal-title":"Blood"},{"key":"e_1_2_10_17_1","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1016\/j.jtct.2022.01.017","article-title":"Outcomes of Allogeneic Hematopoietic Cell Transplantation in T Cell Prolymphocytic Leukemia: A Contemporary Analysis From the Center for International Blood and Marrow Transplant Research","volume":"28","author":"Murthy H. S.","year":"2022","journal-title":"Transplantation and Cellular Therapy"},{"key":"e_1_2_10_18_1","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1038\/s41467-017-02688-6","article-title":"Actionable Perturbations of Damage Responses by TCL1\/ATM and Epigenetic Lesions Form the Basis of T\u2010PLL","volume":"9","author":"Schrader A.","year":"2018","journal-title":"Nature Communications"},{"key":"e_1_2_10_19_1","doi-asserted-by":"crossref","DOI":"10.3389\/fonc.2021.775363","article-title":"Advanced Pathogenetic Concepts in T\u2010Cell Prolymphocytic Leukemia and Their Translational Impact","volume":"11","author":"Braun T.","year":"2021","journal-title":"Frontiers in Oncology"},{"key":"e_1_2_10_20_1","doi-asserted-by":"crossref","first-page":"2786","DOI":"10.1182\/blood.2019003348","article-title":"Noncanonical Effector Functions of the T\u2010Memory\u2010Like T\u2010PLL Cell Are Shaped by Cooperative TCL1A and TCR Signaling","volume":"136","author":"Oberbeck S.","year":"2020","journal-title":"Blood"}],"container-title":["European Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.70312","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ejh.70312","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.70312","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T09:33:36Z","timestamp":1788514416000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ejh.70312"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,4]]},"references-count":19,"alternative-id":["10.1111\/ejh.70312"],"URL":"https:\/\/doi.org\/10.1111\/ejh.70312","archive":["Portico"],"relation":{},"ISSN":["0902-4441","1600-0609"],"issn-type":[{"value":"0902-4441","type":"print"},{"value":"1600-0609","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,4]]},"assertion":[{"value":"2026-04-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-24","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-04","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"ejh.70312"}}