{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T03:43:08Z","timestamp":1789011788889,"version":"build-2803163510"},"reference-count":42,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,4,3]],"date-time":"2023-04-03T00:00:00Z","timestamp":1680480000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>T\u2010cell prolymphocytic leukemia (T\u2010PLL) is a rare, post\u2010thymic T\u2010cell neoplasm with a diverse clinical course. T\u2010PLL is typically associated with a poor prognosis; however, a subset of patients have inactive disease on initial presentation. There is a lack of accurate delineation of the disease based on initial clinical presentation and pathological assessment, hindering clinical decision\u2010making. To characterize and delineate disease subtypes based on initial clinical presentation and pathologic assessment, we retrospectively reviewed 81 patients with T\u2010PLL treated at our institution. We compared patients with T\u2010PLL who initially presented with a relatively indolent or stable disease course to those with an aggressive disease course. Clinicopathologic characteristics, overall survival (OS), and prognostic factors were analyzed. Patients with inactive disease had a significantly longer OS than patients with active disease. At diagnosis, presence of B symptoms, low hemoglobin, low platelet count, lymphocyte doubling time of fewer than 3\u2009months, and abnormal cytogenetics were associated with shorter OS. Cell morphology, immunophenotype, absolute lymphocyte count, lactate dehydrogenase levels, involvement of liver, spleen, skin or central nervous system, presence of <jats:italic>TCL1<\/jats:italic> rearrangement or inv (14)\/t(14;14), presence of chromosome 8 abnormalities, and presence of deletion of 11q were not associated with significant OS difference among the patients. Receiving alemtuzumab as first\u2010line treatment and consolidation with allogeneic hematopoietic stem cell transplant were associated with better outcomes. T\u2010PLL inactive and active disease subtypes can exhibit overlapping yet different clinical and pathological features. We describe several prognostic factors at diagnosis that can be used for risk stratification and aid in guiding treatment decisions.<\/jats:p>","DOI":"10.1002\/ajh.26918","type":"journal-article","created":{"date-parts":[[2023,3,25]],"date-time":"2023-03-25T19:33:19Z","timestamp":1679772799000},"page":"913-921","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Delineation of clinical course, outcomes, and prognostic factors in patients with T\u2010cell prolymphocytic leukemia"],"prefix":"10.1002","volume":"98","author":[{"given":"Ashley","family":"Rose","sequence":"first","affiliation":[{"name":"Department of Malignant Hematology H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ling","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7238-9241","authenticated-orcid":false,"given":"Akriti G.","family":"Jain","sequence":"additional","affiliation":[{"name":"Department of Malignant Hematology H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3083-4834","authenticated-orcid":false,"given":"Abida","family":"Poovathukaran Babu","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine University of South Florida College of Medicine  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lubomir","family":"Sokol","sequence":"additional","affiliation":[{"name":"Department of Malignant Hematology H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hayder","family":"Saeed","sequence":"additional","affiliation":[{"name":"Department of Malignant Hematology H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qianxing","family":"Mo","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Bioinformatics H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenyi","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Bioinformatics H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1033-7550","authenticated-orcid":false,"given":"Xiaohui","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology H. Lee Moffitt Cancer Center and Research Institute  Tampa Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,4,3]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(73)93135-8"},{"key":"e_1_2_10_3_1","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Matutes E","year":"2017"},{"issue":"3","key":"e_1_2_10_4_1","first-page":"538","article-title":"How I treat prolymphocytic leukemia","volume":"120","author":"Dearden C","year":"2012","journal-title":"Blood J Am Soc Hematol"},{"key":"e_1_2_10_5_1","volume-title":"Haematolymphoid Tumours","author":"Elenitoba\u2010Johnson K","year":"2022"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000402"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1998.00977.x"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.13529"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ccr3.1528"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.12788\/jcso.0190"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-01-0002"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24679"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdx163"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V78.12.3269.3269"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-017-02688-6"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.3109\/10428194.2015.1076932"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102007\u201007\u2010101519"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375\u2010020\u20100727\u2010y"},{"key":"e_1_2_10_19_1","first-page":"346","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Matutes E","year":"2017"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0140\u20106736(75)92127\u20103"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19830415)51:8<1434::aid-cncr2820510818>3.0.co;2-4"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V78.12.3269.3269"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.8.3520.bloodjournal8783520"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.10221"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12185\u2010012\u20101077\u2010y"},{"key":"e_1_2_10_26_1","volume-title":"WHO Classification of Tumours Online Haematolymphoid Tumours","author":"Elenitoba\u2010Johnson K","year":"2022"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375\u2010022\u201001620\u20102"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365\u20102141.1998.00977.x"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0165\u20104608(97)00410\u2010x"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/0165-4608(91)90228-M"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.6.2949"},{"issue":"9","key":"e_1_2_10_32_1","first-page":"2475","article-title":"MTCP\u20101: a novel gene on the human chromosome Xq28 translocated to the T cell receptor alpha\/delta locus in mature T cell proliferations","volume":"8","author":"Stern M","year":"1993","journal-title":"Oncogene"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopha.2020.110107"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102014\u201003\u2010559542"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.thj.6200008"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102017\u201009\u2010806398"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102141.1986.tb07579.x"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.12560"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.684621"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102141.1974.tb06769.x"},{"key":"e_1_2_10_41_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.v98.6.1721"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102141.2010.08134.x"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.12430"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.26918","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,19]],"date-time":"2023-08-19T08:02:15Z","timestamp":1692432135000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.26918"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,3]]},"references-count":42,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,6]]}},"alternative-id":["10.1002\/ajh.26918"],"URL":"https:\/\/doi.org\/10.1002\/ajh.26918","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,3]]},"assertion":[{"value":"2023-01-10","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-03-21","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-04-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}