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The 5th WHO edition (Leukemia, 36, 2022 and 1720) classification (WHO\u2010HAEM5) introduced splenic lymphomas\/leukaemias including four different entities: (1) HCL, (2) splenic marginal zone lymphoma (SMZL) with circulating villous cells in the peripheral blood, (3) splenic lymphoma with prominent nucleolus (SLPN), which replaced HCL\u2010V and CD5 negative B\u2010prolymphocytic leukaemia (B\u2010PLL), and (4) splenic diffuse red pulp lymphoma (SDRPL). All these entities have to be distinguished because of a different clinical course and the need for a different treatment. The diagnosis can be challenging because of complex cases and overlap and\/or grey zones between all the entities and needs integrating clinical, histologic, immunophenotypic, cytogenetic and molecular data. We review the diagnostic criteria including clinical, immunophenotypic and molecular characteristics of patients with HCL\u2010V and other HCL\u2010like disorders including HCL, SDRPL, SMZL, B\u2010PLL and the Japanese form of HCL. We also discuss the different criteria allowing us to separate these different entities and we will update the recent therapeutic options that have emerged, in particular the advances with chemoimmunotherapy and\/or targeted therapies.<\/jats:p>","DOI":"10.1111\/jcmm.18060","type":"journal-article","created":{"date-parts":[[2023,12,14]],"date-time":"2023-12-14T12:23:17Z","timestamp":1702556597000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Untangling hairy cell leukaemia (<scp>HCL<\/scp>) variant and other <scp>HCL<\/scp>\u2010like disorders: Diagnosis and treatment"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6863-9992","authenticated-orcid":false,"given":"Xavier","family":"Troussard","sequence":"first","affiliation":[{"name":"H\u00e9matologie CHU Caen Normandie  Caen France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5967-8225","authenticated-orcid":false,"given":"Elsa","family":"Maitre","sequence":"additional","affiliation":[{"name":"H\u00e9matologie CHU Caen Normandie  Caen France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,12,14]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0145-2126(80)90066-1"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01620-2"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022015851"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1974.tb06769.x"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1014209"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26390"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2023.107053"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.3322\/caac.21357"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1521-6926(02)00086-5"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ctarc.2020.100236"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26744"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers14051242"},{"key":"e_1_2_10_14_1","first-page":"57","article-title":"The immunophenotype of hairy cell leukemia (HCL). 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