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Classic HCL displays distinct morphological, immunophenotypical, and genetic features. Classic HCL cells exhibit central nuclei, abundant cytoplasm with hair\u2010like projections, and expression of CD20, CD22, CD11c, CD103, CD25, CD123, TBX21, annexin A1 (ANXA1), FMC7, CD200, and weak cyclin D1 (CCND1). While the vast majority of classic HCL cases harbor the <jats:italic>BRAF<\/jats:italic> V600E somatic mutation, rare examples have been reported without splenomegaly, with bulky lymphadenopathy, or with an atypical morphology, immunophenotype or genotype. This review analyzes the atypical clinical, morphologic, immunophenotypic, and genetic presentations associated with classic HCL. PubMed, Web of Science, and Google Scholar were searched for articles of hairy cell leukemia, including atypical morphology, atypical immunophenotype, atypical genotype, and rare symptoms. Publications from October 2004 to December 2024 were reviewed, with additional relevant studies obtained by reviewing references from selected articles.<\/jats:p>","DOI":"10.1111\/ejh.14388","type":"journal-article","created":{"date-parts":[[2025,2,11]],"date-time":"2025-02-11T02:11:08Z","timestamp":1739239868000},"page":"747-762","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Atypical Hairy Cell Leukemia\u2014The Current Status and Future Directions"],"prefix":"10.1111","volume":"114","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3411-6357","authenticated-orcid":false,"given":"Tadeusz","family":"Robak","sequence":"first","affiliation":[{"name":"Department of Hematology Medical University of Lodz  Lodz Poland"},{"name":"Department of General Hematology Copernicus Memorial Hospital  Lodz Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2082-7086","authenticated-orcid":false,"given":"Marta","family":"Robak","sequence":"additional","affiliation":[{"name":"Department of General Hematology Copernicus Memorial Hospital  Lodz Poland"},{"name":"Department of Hemostasis Disorders Medical University of Lodz  Lodz Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7659-8879","authenticated-orcid":false,"given":"Agata","family":"Majchrzak","sequence":"additional","affiliation":[{"name":"Department of General Hematology Copernicus Memorial Hospital  Lodz Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4670-8306","authenticated-orcid":false,"given":"Anna","family":"Krawczy\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Hematology Medical University of Lodz  Lodz Poland"},{"name":"Department of General Hematology Copernicus Memorial Hospital  Lodz Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3804-7042","authenticated-orcid":false,"given":"Marcin","family":"Braun","sequence":"additional","affiliation":[{"name":"Department of Pathology, Chair of Oncology Medical University of Lodz  Lodz Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,2,10]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-01-689422"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdv200"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/jcmm.18060"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2406376"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.36255\/exon-publications-leukemia-hairy-cell-leukemia"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1014209"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01620-2"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(04)16356-3"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-030-55989-2_12"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.5045\/br.2020.2020069"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.beha.2015.09.002"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ctrv.2010.05.003"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022015851"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-024-02280-0"},{"key":"e_1_2_11_16_1","first-page":"1349","volume-title":"Chapter\u00a084\u2010Hairy Cell Leukaemia","author":"Zakarija A.","year":"2009"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.5045\/br.2017.52.2.139"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12288-014-0442-9"},{"key":"e_1_2_11_19_1","first-page":"1929","volume-title":"Wintrobe's Clinical Hematology","author":"Johnston J. 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