{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T23:38:26Z","timestamp":1779233906651,"version":"3.51.4"},"reference-count":47,"publisher":"Croatian Society for Medical Biochemistry and Laboratory Medicine","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biochem. med. (Online)"],"published-print":{"date-parts":[[2024,6,15]]},"abstract":"<jats:p>Graphical abstract\nHighlights\n\u2022 A chronic lymphoproliferative disease with characteristic mature clonal B lymphocytes with hairy protrusions\n\u2022 Associated with impaired activity of the B-rapidly accelerated fibrosarcoma (BRAF) protein\n\u2022 Differential diagnosis of hairy cell leukemia, hairy cell leukemia variant and splenic marginal zone lymphoma allows for the selection of effective pharmacological therapy and for risk stratification\n\u2022 Therapeutic approach includes purine analogues, BRAF kinase inhibitors, and anticancer immunotherapy\nHairy cell leukemia (HCL) represents 2% of all leukemia cases, with men aged above 55 years being the most affected. The most common symptoms of this type of leukemia include splenomegaly, monocytopenia, and neutropenia. In the basic blood count examination, leukopenia with monocytopenia and granulocytopenia, as well as aplastic anemia and\/or thrombocytopenia occur. The mutation of \u03b2-rapidly accelerated fibrosarcoma (BRAF) proto-oncogene, which can be found in nearly 100% of patients, is an important feature of HCL. Immunophenotypic analysis of the HCL cells reveals high expression of B-lineage antigens, including CD19, CD20, and CD22. Additionally, CD11c, CD25, CD103, and CD123 belong to specific markers of HCL. Lactate dehydrogenase activity and \u03b2-2-microglobulin concentration are also important in the patient\u2019s assessment. The differential diagnosis between HCL, hairy cell leukemia variant (HCL-V) and splenic marginal zone lymphoma (SMZL) is of first importance. Currently, the main treatment for HCL involves the use of purine analogues, excluding pregnant women, individuals with severe infections, and those with relapsing HCL.<\/jats:p>","DOI":"10.11613\/bm.2024.020502","type":"journal-article","created":{"date-parts":[[2024,6,12]],"date-time":"2024-06-12T08:12:53Z","timestamp":1718179973000},"page":"197-209","source":"Crossref","is-referenced-by-count":1,"title":["Hairy cell leukemia \u2013 etiopathogenesis, diagnosis and modern therapeutic approach"],"prefix":"10.11613","volume":"34","author":[{"given":"Iga","family":"Ho\u0142y\u0144ska-Iwan","sequence":"first","affiliation":[{"name":"Department of Pathobiochemistry and Clinical Chemistry, Faculty of Pharmacy, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Toru\u0144, Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karolina","family":"Szewczyk-Golec","sequence":"additional","affiliation":[{"name":"Department of Medical Biology and Biochemistry, Faculty of Medicine, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Toru\u0144, Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katarzyna","family":"Ma\u0107kowiak","sequence":"additional","affiliation":[{"name":"Department of Laboratory Diagnostic, Dr Jan Biziel University Hospital No. 2, Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magdalena","family":"Jankowiak","sequence":"additional","affiliation":[{"name":"Department of Laboratory Diagnostic, Dr Jan Biziel University Hospital No. 2, Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"4885","published-online":{"date-parts":[[2024,6,15]]},"reference":[{"key":"key20240612041227_r1","doi-asserted-by":"crossref","first-page":"7780","DOI":"10.3390\/ijms22157780","article-title":"The biology of Classic Hairy Cell Leukemia.","volume":"22","author":"Bohn","year":"2021","journal-title":"Int J Mol Sci"},{"key":"key20240612041227_r2","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1097\/CCO.0000000000000771","article-title":"Hairy cell leukemia: a brief update on current knowledge and treatment prospects.","volume":"33","author":"Pu\u0142a","year":"2021","journal-title":"Curr Opin Oncol"},{"key":"key20240612041227_r3","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1002\/ajh.26390","article-title":"Hairy cell leukemia 2022: Update on diagnosis, risk-stratification, and treatment.","volume":"97","author":"Troussard","year":"2022","journal-title":"Am J Hematol"},{"key":"key20240612041227_r4","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1111\/bjh.17055","article-title":"on behalf of BSH guidelines committee. 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