{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T18:09:56Z","timestamp":1778695796299,"version":"3.51.4"},"reference-count":35,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2018,3,9]],"date-time":"2018-03-09T00:00:00Z","timestamp":1520553600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2018,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Chronic lymphoproliferative disorder of NK\u2010cells (CLPD\u2010NK) manifests as a persistent increase (\u22652\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L, for &gt; 6\u00a0months) of mature NK\u2010cells in peripheral blood with an indolent clinical course. The disease is rare, and only limited case series have been published.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively studied 11 patients with CLPD\u2010NK diagnosed at our institution between 2005 and 2017.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients included 7 men and 4 women with a median age of 60 years (range, 25\u201089 years). Ten patients (91%) had cytopenias. Bone marrow involvement by CLPD\u2010NK ranged from 5\u201015%. The most commonly detected antigenic aberrancies by \u00a0low cytometry immunophenotyping were as follows: CD7<jats:sup>decreased\/dim<\/jats:sup> (30%), CD8<jats:sup>uniform+<\/jats:sup> (36%), CD56<jats:sup>\u2212\/partial<\/jats:sup> (73%), CD94<jats:sup>bright<\/jats:sup> (55%), and KIR restriction (100%). JAK\/STAT pathway mutations were detected in 8 of 10 (80%) patients and involved <jats:italic>STAT3<\/jats:italic> (n\u00a0=\u00a07) and <jats:italic>JAK3<\/jats:italic> (n\u00a0=\u00a01). The presence of mutations tended to correlate with the occurrence of other cytopenias (anemia\/thrombocytopenia) and requirement for treatment. Seven patients received single\u2010agent therapy, with amelioration of symptoms; 4 patients were observed. There were no disease\u2010associated deaths or progression to more aggressive disease during the follow\u2010up interval (median, 17\u00a0months).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with CLPD\u2010NK have an indolent clinical course and frequent hematologic manifestations that are responsive to single\u2010agent therapy. Mutations in <jats:italic>STAT3<\/jats:italic> are common and portend more pronounced clinical manifestations.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.13038","type":"journal-article","created":{"date-parts":[[2018,1,31]],"date-time":"2018-01-31T22:38:03Z","timestamp":1517438283000},"page":"444-454","source":"Crossref","is-referenced-by-count":23,"title":["Chronic lymphoproliferative disorder of NK\u2010cells: A single\u2010institution review with emphasis on relative utility of multimodality diagnostic tools"],"prefix":"10.1111","volume":"100","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4579-3003","authenticated-orcid":false,"given":"Habibe","family":"Kurt","sequence":"first","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey L.","family":"Jorgensen","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hesham M.","family":"Amin","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keyur P.","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sa A.","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pei","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rashmi","family":"Kanagal\u2010Shamanna","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanam","family":"Loghavi","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5140-3633","authenticated-orcid":false,"given":"Beenu","family":"Thakral","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haitham A.","family":"Khogeer","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elias J.","family":"Jabbour","sequence":"additional","affiliation":[{"name":"Department of Leukemia The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shaoying","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. Cameron","family":"Yin","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L. Jeffrey","family":"Medeiros","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2621-3584","authenticated-orcid":false,"given":"Joseph D.","family":"Khoury","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas MD Anderson Cancer Center  Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,3,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdu369"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0609.2003.00136.x"},{"key":"e_1_2_6_4_1","first-page":"274","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Villamor N","year":"2008"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-03-643544"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-06-435297"},{"key":"e_1_2_6_7_1","first-page":"599","volume-title":"Hematopathology","author":"Morice WG","year":"2017"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.1.268"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAP.0b013e3181916029"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-17-1597"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/cyto.b.20363"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/cyto.b.21518"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1053\/S0046-8177(03)00412-X"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1159\/isbn.978-3-318-05979-3"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2011.158"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2013.093765"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2012.88"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/85820"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1309\/AJCPD7NR2RMNQDVF"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1309\/5WFQ-N12E-DT05-UX1T"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13039-015-0171-2"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1309\/Q49CRJ030L22MHLF"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2009.304"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/377065a0"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.270.5237.797"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-12-0028"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2013.157"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1114885"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1203476"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-1119(02)00398-0"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1205398"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-12-0191"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aac5272"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/cas.12683"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-12-0284"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2015.280"}],"container-title":["European Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fejh.13038","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.13038","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T16:04:53Z","timestamp":1695571493000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ejh.13038"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,3,9]]},"references-count":35,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2018,5]]}},"alternative-id":["10.1111\/ejh.13038"],"URL":"https:\/\/doi.org\/10.1111\/ejh.13038","archive":["Portico"],"relation":{},"ISSN":["0902-4441","1600-0609"],"issn-type":[{"value":"0902-4441","type":"print"},{"value":"1600-0609","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,3,9]]}}}