{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T18:50:27Z","timestamp":1789498227117,"version":"build-2803163510"},"reference-count":163,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2024,6,10]],"date-time":"2024-06-10T00:00:00Z","timestamp":1717977600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Introduction: Hairy-cell leukemia (HCL) is a rare B-cell chronic lymphoproliferative disorder (B-CLPD), whose favorable prognosis has changed with the use of purine nucleoside analogs (PNAs), such as cladribine (CDA) or pentostatin (P). However, some patients eventually relapse and over time HCL becomes resistant to chemotherapy. Many discoveries have been made in the pathophysiology of HCL during the last decade, especially in genomics, with the identification of the BRAFV600E mutation and cellular biology, including the importance of signaling pathways as well as tumor microenvironment. All of these new developments led to targeted treatments, especially BRAF inhibitors (BRAFis), MEK inhibitors (MEKis), Bruton\u2019s tyrosine kinase (BTK) inhibitors (BTKis) and recombinant anti-CD22 immunoconjugates. Results: The following major changes or additions were introduced in these updated guidelines: the clinical relevance of the changes in the classification of splenic B-cell lymphomas and leukemias; the increasingly important diagnostic role of BRAFV600E mutation; and the prognostic role of the immunoglobulin (IG) variable (V) heavy chain (H) (IGHV) mutational status and repertory. We also wish to insist on the specific involvement of bones, skin, brain and\/or cerebrospinal fluid (CSF) of the disease at diagnosis or during the follow-up, the novel targeted drugs (BRAFi and MEKi) used for HCL treatment, and the increasing role of minimal residual disease (MRD) assessment. Conclusion: Here we present recommendations for the diagnosis of HCL, treatment in first line and in relapsed\/refractory patients as well as for HCL-like disorders including HCL variant (HCL-V)\/splenic B-cell lymphomas\/leukemias with prominent nucleoli (SBLPN) and splenic diffuse red pulp lymphoma (SDRPL).<\/jats:p>","DOI":"10.3390\/cancers16122185","type":"journal-article","created":{"date-parts":[[2024,6,10]],"date-time":"2024-06-10T08:59:06Z","timestamp":1718009946000},"page":"2185","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group"],"prefix":"10.3390","volume":"16","author":[{"given":"J\u00e9r\u00f4me","family":"Paillassa","sequence":"first","affiliation":[{"name":"Service des Maladies du Sang, CHU d\u2019Angers, 49000 Angers, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5967-8225","authenticated-orcid":false,"given":"Elsa","family":"Maitre","sequence":"additional","affiliation":[{"name":"H\u00e9matologie Biologique, Structure F\u00e9d\u00e9rative D\u2019oncog\u00e9n\u00e9tique Cyto-Mol\u00e9culaire du CHU de Caen (SF-MOCAE), CHU de Caen, 14000 Caen, France"},{"name":"Unit\u00e9 MICAH, INSERM1245, Universit\u00e9 Caen-Normandie, 14000 Caen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Belarbi Boudjerra","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie, CHU B\u00e9ni Messous, Alger 16308, Algeria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdallah","family":"Madani","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie, CHU de Casablanca, Casablanca 20000, Morocco"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raihane","family":"Benlakhal","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie, CHU Aziza Othmena, Tunis 1002, Tunisia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4875-2477","authenticated-orcid":false,"given":"Thomas","family":"Matthes","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie, D\u00e9partement d\u2019Oncologie et Service de Pathologie Clinique, D\u00e9partement de Diagnostic, H\u00f4pital Universitaire de Gen\u00e8ve, 1205 Gen\u00e8ve, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Van Den Neste","sequence":"additional","affiliation":[{"name":"Cliniques Universitaires Saint-Luc, Universit\u00e9 Catholique de Louvain, 1000 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Cailly","sequence":"additional","affiliation":[{"name":"Service d\u2019Onco-H\u00e9matologie et de Th\u00e9rapie Cellulaire, CHU de Poitiers, 86000 Poitiers, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Inchiappa","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie, Institut Paoli-Calmette, 13397 Marseille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed Amine","family":"Bekadja","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie et de Th\u00e9rapie Cellulaire, EHU Oran, Oran 31000, Algeria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4093-6463","authenticated-orcid":false,"given":"C\u00e9cile","family":"Tomowiak","sequence":"additional","affiliation":[{"name":"Service d\u2019Onco-H\u00e9matologie et de Th\u00e9rapie Cellulaire, CHU de Poitiers, 86000 Poitiers, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Troussard","sequence":"additional","affiliation":[{"name":"H\u00e9matologie Biologique, Structure F\u00e9d\u00e9rative D\u2019oncog\u00e9n\u00e9tique Cyto-Mol\u00e9culaire du CHU de Caen (SF-MOCAE), CHU de Caen, 14000 Caen, France"},{"name":"Hematologie CHU Caen Normandie, 14000 Caen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1182\/blood.V13.7.609.609","article-title":"Leukemic reticuloendotheliosis","volume":"13","author":"Bouroncle","year":"1958","journal-title":"Blood"},{"key":"ref_2","first-page":"57","article-title":"The immunophenotype of hairy cell leukemia (HCL). 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