{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T22:57:48Z","timestamp":1773961068632,"version":"3.50.1"},"reference-count":29,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,6,9]],"date-time":"2020-06-09T00:00:00Z","timestamp":1591660800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","award":["KL2TR002734"],"award-info":[{"award-number":["KL2TR002734"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>T\u2010cell large granular lymphocytic leukaemia (T\u2010LGLL) is an incurable leukaemia characterised by clonal proliferation of abnormal cytotoxic T cells that can result in severe neutropenia, transfusion\u2010dependent anaemia and pancytopenia requiring treatment. The most commonly used agents, methotrexate (MTX), cyclophosphamide (Cy) and cyclosporine primarily produce partial remissions (PRs), with few complete responses (CRs). We evaluated the clinical course and treatment response of 60 consecutive patients with T\u2010LGLL to evaluate clinical outcomes and future potential treatment directions. Impaired overall survival was noted among male patients, patients with elevated lactate dehydrogenase, and those without rheumatoid arthritis. Cy was the most efficacious second\u2010line agent, with a 70% overall response rate (ORR; three CR, four PR). All patients who failed frontline MTX responded to second\u2010line Cy. In the relapsed or Cy\u2010refractory setting, alemtuzumab (<jats:italic>n<\/jats:italic>\u00a0=\u00a04) and pentostatin (<jats:italic>n<\/jats:italic>\u00a0=\u00a03) had an ORR of 50% and 66%, respectively, while duvelisib induced a long\u2010term response in one patient. In this large, retrospective analysis, our results suggest Cy is a highly effective therapy for second\u2010line treatment in T\u2010LGLL and should be considered a strong candidate for up\u2010front therapy in select high\u2010risk patients. Prospective studies evaluating pentostatin, alemtuzumab and novel agents, such as duvelisib, are needed for patients with relapsed\/refractory T\u2010LGLL.<\/jats:p>","DOI":"10.1111\/bjh.16808","type":"journal-article","created":{"date-parts":[[2020,6,10]],"date-time":"2020-06-10T00:19:12Z","timestamp":1591748352000},"page":"484-493","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Clinical outcomes in T\u2010cell large granular lymphocytic leukaemia: prognostic factors and treatment response"],"prefix":"10.1111","volume":"192","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2517-9943","authenticated-orcid":false,"given":"Zachary","family":"Braunstein","sequence":"first","affiliation":[{"name":"Department of Internal Medicine The Ohio State University Columbus OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anjali","family":"Mishra","sequence":"additional","affiliation":[{"name":"Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation Department of Medical Oncology and Department of Cancer Biology Sydney Kimmel Cancer Center Thomas Jefferson University Philadelphia PA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annette","family":"Staub","sequence":"additional","affiliation":[{"name":"Division of Nursing James Comprehensive Cancer Center The Ohio State University Columbus OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aharon G.","family":"Freud","sequence":"additional","affiliation":[{"name":"Department of Pathology James Comprehensive Cancer Center The Ohio State University Columbus OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierluigi","family":"Porcu","sequence":"additional","affiliation":[{"name":"Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation Department of Medical Oncology Sydney Kimmel Cancer Center Thomas Jefferson University Philadelphia PA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jonathan E.","family":"Brammer","sequence":"additional","affiliation":[{"name":"Division of Hematology Department of Internal Medicine James Comprehensive Cancer Center The Ohio State University Columbus OH 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