{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T04:33:27Z","timestamp":1745469207562,"version":"3.40.4"},"reference-count":14,"publisher":"S. Karger AG","issue":"2","license":[{"start":{"date-parts":[[2016,6,10]],"date-time":"2016-06-10T00:00:00Z","timestamp":1465516800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2016,6,10]],"date-time":"2016-06-10T00:00:00Z","timestamp":1465516800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Rep Oncol"],"abstract":"<jats:p>A 65-year-old woman presented with easy bruising, left upper quadrant pain, decreased appetite, and weight loss. She had splenomegaly and lymphocytosis (lymphocyte count of 11.6 \u00d7 10&lt;sup&gt;9&lt;\/sup&gt;\/l), with remarkably abnormal appearing morphology. Her hemoglobin and platelet counts were normal. Peripheral blood flow cytometry revealed a monoclonal B-cell population expressing CD11c, CD25, CD19, CD20, and CD103. An initial diagnosis of hairy cell leukemia (HCL) was made, and the patient was treated with a standard 5-day course of cladribine. However, her lymphocytosis improved transiently, with a relapse 4 months later. There was no improvement in her splenomegaly. An HCL variant (HCL-v) was considered based on her resistance to treatment with a purine nucleoside analog. A subsequent splenectomy improved symptoms. Two years after, the patient suffered a relapse and underwent 6 cycles of CHOP-R (cyclophosphamide, hydroxydaunomycin, oncovin, prednisone, and rituximab), achieving partial remission. While under observation, she progressed with lymphocytosis 6 months later and was treated with pentostatin. There was no significant improvement in her disease, and she died 8 weeks following treatment initiation. HCL-v is a clinically more aggressive mature B-cell lymphoma than HCL with worse splenomegaly, higher lymphocyte counts, and resistance to typical HCL therapy with purine nucleoside analogs. Early recognition of HCL-v in the history, physical examination, and investigations with morphology and flow cytometry is key to patient management. Further, as in our case of HCL-v, cell morphology can be distinctly atypical, with large nucleoli and extremely convoluted nuclei. The distinction between HCL and HCL-v is important as HCL-v patients require more aggressive therapy and closer follow-up.<\/jats:p>","DOI":"10.1159\/000446696","type":"journal-article","created":{"date-parts":[[2016,6,10]],"date-time":"2016-06-10T23:48:24Z","timestamp":1465602504000},"page":"312-316","source":"Crossref","is-referenced-by-count":1,"title":["A Unique Hairy Cell Leukemia Variant"],"prefix":"10.1159","volume":"9","author":[{"given":"Charles","family":"Jian","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cyrus\u00a0C.","family":"Hsia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2016,6,10]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2011.09.001"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1007\/s11864-014-0285-5"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.7860\/JCDR\/2013\/5169.2768"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.bjhh.2015.01.003"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.ctrv.2006.04.010"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016\/S1521-6926(02)00086-5"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2401999"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2006.06.012"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1309\/8QYTYQ1CLQMHQ9CL"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-379339"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-06-195370"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200107263450402"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12867"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1506583"}],"container-title":["Case Reports in Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/446696","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/446696","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/446696","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T18:10:30Z","timestamp":1745431830000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000446696"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,6,10]]},"references-count":14,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2016,9,7]]}},"URL":"https:\/\/doi.org\/10.1159\/000446696","archive":["Portico"],"relation":{},"ISSN":["1662-6575"],"issn-type":[{"type":"electronic","value":"1662-6575"}],"subject":[],"published":{"date-parts":[[2016,6,10]]}}}