{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T17:12:53Z","timestamp":1780593173793,"version":"3.54.1"},"reference-count":38,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2023,3,8]],"date-time":"2023-03-08T00:00:00Z","timestamp":1678233600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,4,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>T-cell prolymphocytic leukemia (T-PLL) is a rare mature T-cell leukemia usually characterized by inv(14)(q11.2q32)\/t(14;14)(q11.2;q32). In this study, we aimed to investigate the clinicopathologic features and molecular profile of T-PLL associated with t(X;14)(q28;q11.2).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The study group included 10 women and 5 men with a median age of 64 years. All 15 patients had a diagnosis of T-PLL with t(X;14)(q28;q11.2).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All 15 patients had lymphocytosis at initial diagnosis. Morphologically, the leukemic cells had features of prolymphocytes in 11 patients, small cell variant in 3, and cerebriform variant in 1. All 15 patients had hypercellular bone marrow with an interstitial infiltrate in 12 (80%) cases. By flow cytometry, the leukemic cells were surface CD3+\/CD5+\/CD7+\/CD26+\/CD52+\/TCR \u03b1\/\u03b2+\u2005in 15 (100%) cases, CD2+\u2005in 14 (93%) cases, CD4+\/CD8+\u2005in 8 (53%) cases, CD4+\/CD8\u2013 in 6 (40%) cases, and CD4\u2013\/CD8\u2005+\u2005in 1 (7%) case. At the cytogenetic level, complex karyotypes with t(X;14)(q28;q11.2) were seen in all 15 patients assessed. Mutational analysis showed mutations of JAK3 in 5 of 6 and STAT5B p.N642H in 2 of 6 patients. Patients received variable treatments, including 12 with alemtuzumab. After a median follow-up of 17.2 months, 8 of 15 (53%) patients died.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>T-PLL with t(X;14)(q28;q11.2) frequently shows a complex karyotype and mutations involving JAK\/STAT pathway, and it is an aggressive disease with a poor outcome.<\/jats:p><\/jats:sec>","DOI":"10.1093\/ajcp\/aqac166","type":"journal-article","created":{"date-parts":[[2023,3,8]],"date-time":"2023-03-08T13:43:23Z","timestamp":1678283003000},"page":"325-336","source":"Crossref","is-referenced-by-count":17,"title":["T-Cell Prolymphocytic Leukemia With t(X;14)(q28;q11.2): A Clinicopathologic Study of 15 Cases"],"prefix":"10.1093","volume":"159","author":[{"given":"Zhihong","family":"Hu","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine, The University of Texas MD Anderson Cancer Center , Houston, TX , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L Jeffrey","family":"Medeiros","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mina","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Yale University Medical Center , New Haven, CT , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji","family":"Yuan","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine & Pathology, Mayo Clinic , Minneapolis, MN , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deniz","family":"Peker","sequence":"additional","affiliation":[{"name":"Department of Pathology, Emory University , Atlanta, GA , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina","family":"Shao","sequence":"additional","affiliation":[{"name":"Department of Pathology, The University of Michigan Medical Center , Ann Arbor, MI , USA and"},{"name":"Departments of , Ann Arbor, MI , USA and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhenya","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brenda","family":"Mai","sequence":"additional","affiliation":[{"name":"Department of Pathology, The University of Texas Health Science Center at Houston, Houston, TX, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beenu","family":"Thakral","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adan","family":"Rios","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, The University of Texas Health Science Center at Houston, Houston, TX, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shimin","family":"Hu","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Hematopathology, The University of Texas MD Anderson Cancer Center , Houston, TX , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,3,8]]},"reference":[{"key":"2023040406404441600_","first-page":"176","article-title":"T lymphoblastic leukaemia\/lymphoma.","volume-title":"WHO Classifications of Tumours of Haematopoietic and Lymphoid Tissues","author":"Borowitz","year":"2008"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1002\/ajh.24679","article-title":"Prognostic significance of cytogenetic abnormalities in T-cell prolymphocytic leukemia","volume":"92","author":"Hu","year":"2017","journal-title":"Am J Hematol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"e17","DOI":"10.1111\/ijlh.13662","article-title":"Incidental but rapidly progressing T-cell prolymphocytic leukemia with t(X;14)(q28;q11) involving parotid lymphoepithelial cysts: a diagnostic pitfall.","volume":"44","author":"Ghosh","year":"2022","journal-title":"Int J Lab Hematol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/S0165-4608(97)00410-X","article-title":"Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization","volume":"103","author":"Maljaei","year":"1998","journal-title":"Cancer Genet Cytogenet"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1002\/ijc.2910340206","article-title":"Chromosome studies in patients with T-CLL chronic lymphocytic leukemia and expansions of granular lymphocytes","volume":"34","author":"Dallapiccola","year":"1984","journal-title":"Int J Cancer"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1046\/j.1365-2141.2000.02256.x","article-title":"T-cell prolymphocytic leukaemia: antigen receptor gene rearrangement and a novel mode of MTCP1 B1 activation","volume":"110","author":"De Schouwer","year":"2000","journal-title":"Br J Haematol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/0165-4608(89)90085-X","article-title":"Twenty-six patients with hematologic disorders and X chromosome abnormalities: frequent idic(X)(q13) chromosomes and Xq13 anomalies associated with pathologic ringed sideroblasts","volume":"42","author":"Dewald","year":"1989","journal-title":"Cancer Genet Cytogenet"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/S0925-5710(98)00074-7","article-title":"14q11 abnormality and trisomy 8q are not common in Japanese T-cell prolymphocytic leukemia","volume":"68","author":"Kojima","year":"1998","journal-title":"Int J Hematol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1111\/j.1751-553X.2008.01036.x","article-title":"Translocations t(X;14)(q28;q11) and t(Y;14)(q12;q11) in T-cell prolymphocytic leukemia","volume":"31","author":"de Oliveira","year":"2009","journal-title":"Int J Lab Hematol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1182\/blood.2019000402","article-title":"Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia","volume":"134","author":"Staber","year":"2019","journal-title":"Blood"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"1720","DOI":"10.1038\/s41375-022-01620-2","article-title":"The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms","volume":"36","author":"Alaggio","year":"2022","journal-title":"Leukemia"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1002\/gcc.22821","article-title":"Reconstruction of rearranged T-cell receptor loci by whole genome and transcriptome sequencing gives insights into the initial steps of T-cell prolymphocytic leukemia","volume":"59","author":"Patil","year":"2020","journal-title":"Genes Chromosomes Cancer"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1309\/AJCPG71KYOXTKLQW","article-title":"Immunophenotypic characterization of T-cell prolymphocytic leukemia","volume":"140","author":"Chen","year":"2013","journal-title":"Am J Clin Pathol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1136\/jclinpath-2017-204616","article-title":"Comparison of karyotyping, TCL1 fluorescence in situ hybridisation and TCL1 immunohistochemistry in T cell prolymphocytic leukaemia","volume":"71","author":"Sun","year":"2018","journal-title":"J Clin Pathol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1002\/cyto.b.21782","article-title":"Single antibody detection of T-cell receptor alphabeta clonality by flow cytometry rapidly identifies mature T-cell neoplasms and monotypic small CD8-positive subsets of uncertain significance","volume":"98","author":"Shi","year":"2020","journal-title":"Cytometry B Clin Cytom"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"2046","DOI":"10.1038\/s41379-020-0568-2","article-title":"T-cell clones of uncertain significance are highly prevalent and show close resemblance to T-cell large granular lymphocytic leukemia: implications for laboratory diagnostics","volume":"33","author":"Shi","year":"2020","journal-title":"Mod Pathol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1111\/bjh.13952","article-title":"Genes encoding members of the JAK-STAT pathway or epigenetic regulators are recurrently mutated in T-cell prolymphocytic leukaemia","volume":"173","author":"Lopez","year":"2016","journal-title":"Br J Haematol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"1833","DOI":"10.3390\/cancers11121833","article-title":"JAK\/STAT-activating genomic alterations are a hallmark of T-PLL.","volume":"11","author":"Wahnschaffe","year":"2019","journal-title":"Cancers (Basel)"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1038\/leu.2013.271","article-title":"Recurrent JAK1 and JAK3 somatic mutations in T-cell prolymphocytic leukemia","volume":"28","author":"Bellanger","year":"2014","journal-title":"Leukemia"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1002\/gcc.22141","article-title":"Recurrent mutation of JAK3 in T-cell prolymphocytic leukemia","volume":"53","author":"Bergmann","year":"2014","journal-title":"Genes Chromosomes Cancer"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"1460","DOI":"10.1182\/blood-2014-03-559542","article-title":"Integrated genomic sequencing reveals mutational landscape of T-cell prolymphocytic leukemia","volume":"124","author":"Kiel","year":"2014","journal-title":"Blood"},{"key":"2023040406404441600_","first-page":"2415","article-title":"JAK3: a novel JAK kinase associated with terminal differentiation of hematopoietic cells","volume":"9","author":"Rane","year":"1994","journal-title":"Oncogene"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1186\/gb-2004-5-12-253","article-title":"The Janus kinases (Jaks)","volume":"5","author":"Yamaoka","year":"2004","journal-title":"Genome Biol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"3996","DOI":"10.1182\/blood.V90.10.3996","article-title":"Structural and functional basis for JAK3-deficient severe combined immunodeficiency","volume":"90","author":"Candotti","year":"1997","journal-title":"Blood"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1126\/science.270.5237.797","article-title":"Mutation of Jak3 in a patient with SCID: essential role of Jak3 in lymphoid development","volume":"270","author":"Russell","year":"1995","journal-title":"Science"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1038\/leu.2016.13","article-title":"JAK-STAT and G-protein-coupled receptor signaling pathways are frequently altered in epitheliotropic intestinal T-cell lymphoma","volume":"30","author":"Nairismagi","year":"2016","journal-title":"Leukemia"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1038\/leu.2013.157","article-title":"JAK3 deregulation by activating mutations confers invasive growth advantage in extranodal nasal-type natural killer cell lymphoma","volume":"28","author":"Bouchekioua","year":"2014","journal-title":"Leukemia"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"3911","DOI":"10.1182\/blood-2010-12-319467","article-title":"FERM domain mutations induce gain of function in JAK3 in adult T-cell leukemia\/lymphoma","volume":"118","author":"Elliott","year":"2011","journal-title":"Blood"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1002\/jcp.10364","article-title":"STAT proteins: from normal control of cellular events to tumorigenesis","volume":"197","author":"Calo","year":"2003","journal-title":"J Cell Physiol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1038\/nrm909","article-title":"Stats: transcriptional control and biological impact","volume":"3","author":"Levy","year":"2002","journal-title":"Nat Rev Mol Cell Biol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1200\/JCO.2004.10.124","article-title":"The role of signal transducer and activator of transcription factors in leukemogenesis","volume":"22","author":"Sternberg","year":"2004","journal-title":"J Clin Oncol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1038\/leu.2017.252","article-title":"Discovery of novel drug sensitivities in T-PLL by high-throughput ex vivo drug testing and mutation profiling","volume":"32","author":"Andersson","year":"2018","journal-title":"Leukemia"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1002\/gcc.22313","article-title":"Genetic characterization of T-PLL reveals two major biologic subgroups and JAK3 mutations as prognostic marker","volume":"55","author":"Stengel","year":"2016","journal-title":"Genes Chromosomes Cancer"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1182\/blood-2012-01-380139","article-title":"How I treat prolymphocytic leukemia","volume":"120","author":"Dearden","year":"2012","journal-title":"Blood"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1182\/asheducation.V2015.1.361.3917560","article-title":"Management of prolymphocytic leukemia","volume":"2015","author":"Dearden","year":"2015","journal-title":"Hematology Am Soc Hematol Educ Program"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1182\/blood-2004-01-0002","article-title":"A systematic approach to diagnosis of mature T-cell leukemias reveals heterogeneity among WHO categories","volume":"104","author":"Herling","year":"2004","journal-title":"Blood"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1111\/j.1365-2141.2010.08134.x","article-title":"Stem cell transplantation after alemtuzumab in T-cell prolymphocytic leukaemia results in longer survival than after alemtuzumab alone: a multicentre retrospective study","volume":"149","author":"Krishnan","year":"2010","journal-title":"Br J Haematol"},{"key":"2023040406404441600_","doi-asserted-by":"crossref","first-page":"972","DOI":"10.1038\/leu.2011.304","article-title":"Hematopoietic stem cell transplantation in T-prolymphocytic leukemia: a retrospective study from the European Group for Blood and Marrow Transplantation and the Royal Marsden Consortium","volume":"26","author":"Wiktor-Jedrzejczak","year":"2012","journal-title":"Leukemia"}],"container-title":["American Journal of Clinical Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ajcp\/article-pdf\/159\/4\/325\/49737923\/aqac166.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ajcp\/article-pdf\/159\/4\/325\/49737923\/aqac166.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,12,2]],"date-time":"2023-12-02T21:13:22Z","timestamp":1701551602000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ajcp\/article\/159\/4\/325\/7072566"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,8]]},"references-count":38,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2023,3,8]]},"published-print":{"date-parts":[[2023,4,4]]}},"URL":"https:\/\/doi.org\/10.1093\/ajcp\/aqac166","relation":{},"ISSN":["0002-9173","1943-7722"],"issn-type":[{"value":"0002-9173","type":"print"},{"value":"1943-7722","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,4,1]]},"published":{"date-parts":[[2023,3,8]]}}}