{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,13]],"date-time":"2025-10-13T01:24:06Z","timestamp":1760318646500},"reference-count":20,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2002,3,22]],"date-time":"2002-03-22T00:00:00Z","timestamp":1016755200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Comiss\u00e3o de Fomento da Investiga\u00e7\u00e3o em Cuidados de Sa\u00fade."},{"name":"Minist\u00e9rio da Educa\u00e7\u00e3o, Portugal;","award":["PI 51\/99."],"award-info":[{"award-number":["PI 51\/99."]}]},{"name":"Ac\u00e7\u00f5es Integradas Luso-Espanholas do Conselho de Reitores das Universidades Portuguesas;","award":["E-31\/99."],"award-info":[{"award-number":["E-31\/99."]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematological Oncology"],"published-print":{"date-parts":[[2002,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We report a case of CD8<jats:sup>+<\/jats:sup>\/V\u03b25.1<jats:sup>+<\/jats:sup> T\u2010cell large granular lymphocyte leukemia (T\u2010LGL leukemia) presenting with mild lymphocytosis, severe autoimmune neutropenia, thrombocytopenia, polyarthritis and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3<jats:sup>+<\/jats:sup>\/TCR\u03b1\u03b2<jats:sup>+<\/jats:sup>\/CD8<jats:sup>+bright<\/jats:sup>\/CD11c<jats:sup>+<\/jats:sup>\/CD57<jats:sup>\u2212<\/jats:sup>\/CD56<jats:sup>\u2212<\/jats:sup> large granular lymphocytes with expression of the TCR\u2010V\u03b25.1 family. Southern blot analysis revealed a clonal rearrangement of the TCR \u03b2\u2010chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin and corticosteroids were of limited therapeutic benefit to correct the cytopenias. During the disease course, the patient developed a severe cutaneous leg ulcer and bilateral vascular mammary skin lesions. Treatment with 2\u2010deoxycoformycin resulted in both clinical and hematological complete responses, including the resolution of vascular skin lesions. Combined immuno\u2010staining with relevant T\u2010cell associated and anti\u2010TCR\u2010V\u03b2 monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2\u2010deoxycoformicin and to evaluate the residual disease. Copyright \u00a9 2002 John Wiley &amp; Sons, Ltd.<\/jats:p>","DOI":"10.1002\/hon.695","type":"journal-article","created":{"date-parts":[[2002,8,25]],"date-time":"2002-08-25T23:29:46Z","timestamp":1030318186000},"page":"87-93","source":"Crossref","is-referenced-by-count":12,"title":["Cd8<sup>+<\/sup>\/v\u03b25.1<sup>+<\/sup> large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2\u2010deoxycoformycin."],"prefix":"10.1002","volume":"20","author":[{"given":"E.","family":"Granjo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Lima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Correia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Lisboa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Magalh\u00e3es","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Cunha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"MA.","family":"Teixeira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"ML.","family":"Queir\u00f3s","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Candeias","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Matutes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2002,3,22]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0268-960X(92)90018-L"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V82.1.1.bloodjournal8211"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(94)70157-1"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01695691"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9343(00)00406-X"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1159\/000040906"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V91.9.3372"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)61377-9"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830490309"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199704)54:4<329::AID-AJH13>3.0.CO;2-6"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1994.12.12.2588"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199803)57:3<253::AID-AJH14>3.0.CO;2-B"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01525510"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1128\/CDLI.5.5.675-682.1998"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.163.2.553"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.3109\/08916939409009529"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1177\/030089169408000412"},{"key":"e_1_2_1_19_2","first-page":"297","article-title":"Mesangiolysis associated with severe glomerular endocapillary proliferation of CD57 large granular lymphocytes","volume":"43","author":"Hotta O","year":"1995","journal-title":"Clin Nephrol"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1038\/nm0498-408"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.138.2.274"}],"container-title":["Hematological Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhon.695","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/hon.695","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,18]],"date-time":"2023-11-18T09:13:09Z","timestamp":1700298789000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/hon.695"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,3,22]]},"references-count":20,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2002,6]]}},"alternative-id":["10.1002\/hon.695"],"URL":"https:\/\/doi.org\/10.1002\/hon.695","archive":["Portico"],"relation":{},"ISSN":["0278-0232","1099-1069"],"issn-type":[{"value":"0278-0232","type":"print"},{"value":"1099-1069","type":"electronic"}],"subject":[],"published":{"date-parts":[[2002,3,22]]}}}