{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T13:35:48Z","timestamp":1790516148120,"version":"4.1.0"},"reference-count":39,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,7,12]],"date-time":"2021-07-12T00:00:00Z","timestamp":1626048000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Reports"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hairy cell leukemia (HCL) is a rare chronic B\u2010cell neoplasm with good long\u2010term prognosis. First and second\u2010line therapies include purine nucleoside analogues (PNAs) and rituximab, but until recently, limited alternative options were available for patients with two or more relapses.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>The aim of this study is to describe our real\u2010life experience with HCL patients in third and fourth\u2010line therapies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and Results<\/jats:title><jats:p>Data from 49 HCL patients with two or more relapses, including 16 patients with three or more relapses, were collected from the French retrospective HCL cohort covering the period from 1980 until 2011. They were analyzed to assess hematological response, relapse free survival (RFS) and overall survival (OS) after third (L3) and fourth line (L4). The median age at diagnosis was 53\u2009years. PNAs were the most frequently used treatments. As L3 therapy, 29 patients received PNAs (66%) and 15 (34%) other treatments (rituximab [11%] or interferon [7%] alone or in combination [16%]). The distribution of L4 treatments was similar. The overall hematological response rate (OHRR) after L3 was 97% (complete hematological response 86%) with a 40% five\u2010year cumulative incidence of relapse (CIR), a median RFS of 104\u2009months, and a median OS of 235\u2009months. After L4, the OHRR was 94% with a two\u2010year CIR of fourth relapse of 27%. Eleven secondary cancers (5\u2010year cumulative incidence of 12%) were diagnosed in 10 patients. Patients with \u22652 relapses experience frequent further relapses, with increasingly shorter time to next treatment as the number of treatment lines increases. Furthermore, treatment strategies are associated with substantial toxicities.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>All these points lead to the need for novel treatments.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cnr2.1495","type":"journal-article","created":{"date-parts":[[2021,7,12]],"date-time":"2021-07-12T07:36:01Z","timestamp":1626075361000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Patients with relapsed\/refractory hairy\u2010cell leukemia"],"prefix":"10.1002","volume":"5","author":[{"given":"J\u00e9r\u00f4me","family":"Paillassa","sequence":"first","affiliation":[{"name":"Department of Hematology Academic Hospital of Angers  Angers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6863-9992","authenticated-orcid":false,"given":"Xavier","family":"Troussard","sequence":"additional","affiliation":[{"name":"Laboratory of Hematology Academic Hospital of Caen  Caen France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,7,12]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1999.17.12.3835"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V13.7.609.609"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V27.2.199.199"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(18)30609-0"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1014209"},{"key":"e_1_2_10_7_1","article-title":"Estimations nationales de l'incidence et de la mortalit\u00e9 par cancer en France m\u00e9tropolitaine entre 1990 et 2018.","volume":"2","author":"Le Guyader\u2010Peyro S","year":"2019","journal-title":"H\u00e9mopathies malignes"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.31.7016"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198704023161401"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199004193221605"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1995.13.4.974"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1998.16.9.3007"},{"issue":"9","key":"e_1_2_10_13_1","first-page":"2981","article-title":"Long\u2010term follow\u2010up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin","volume":"96","author":"Flinn IW","year":"2000","journal-title":"Blood"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2003.05.093"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2009.07668.x"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.25243"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.beha.2015.09.004"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11864-020-00732-0"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12908"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-014-2140-y"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-03-643544"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41408-020-0328-z"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clml.2017.07.003"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V92.6.1918"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2402784"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1080\/10428199909169620"},{"issue":"4","key":"e_1_2_10_27_1","first-page":"291","article-title":"Retrospective evaluation of hairy cell leukemia patients treated with three different first\u2010line treatment modalities in the last two decades: a single\u2010center experience","volume":"34","author":"\u00d6ng\u00f6ren \u015e","year":"2017","journal-title":"Turkish J Haematol Off J Turkish Soc Haematol"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2403418"},{"issue":"3","key":"e_1_2_10_29_1","first-page":"575","article-title":"Hairy cell leukemia\u2013a rare type of leukemia. 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