{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,8]],"date-time":"2025-12-08T22:09:07Z","timestamp":1765231747349},"reference-count":42,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2008,5,12]],"date-time":"2008-05-12T00:00:00Z","timestamp":1210550400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"},{"start":{"date-parts":[[2008,5,12]],"date-time":"2008-05-12T00:00:00Z","timestamp":1210550400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                        <jats:title>Introduction<\/jats:title>\n                        <jats:p>The purpose of this study was to analyze the data of patients with T-cell large granular lymphocyte (T-LGL) lymphocytosis associated with inflammatory arthropathy or with no arthritis symptoms.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Methods<\/jats:title>\n                        <jats:p>Clinical, serological as well as histopathological, immuhistochemical, and flow cytometric evaluations of blood\/bone marrow of 21 patients with T-LGL lymphocytosis were performed. The bone marrow samples were also investigated for T-cell receptor (<jats:italic>TCR<\/jats:italic>) and immunoglobulin (<jats:italic>IG<\/jats:italic>) gene rearrangements by polymerase chain reaction with heteroduplex analysis.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Results<\/jats:title>\n                        <jats:p>Neutropenia was observed in 21 patients, splenomegaly in 10, autoimmune diseases such as rheumatoid arthritis (RA) in 9, unclassified arthritis resembling RA in 2, and autoimmune thyroiditis in 5 patients. T-LGL leukemia was recognized in 19 cases. Features of Felty syndrome were observed in all RA patients, representing a spectrum of T-LGL proliferations from reactive polyclonal through transitional between reactive and monoclonal to T-LGL leukemia. Bone marrow trephines from T-LGL leukemia patients showed interstitial clusters and intrasinusoidal linear infiltrations of CD3<jats:sup>+<\/jats:sup>\/CD8<jats:sup>+<\/jats:sup>\/CD57<jats:sup>+<\/jats:sup>\/granzyme B<jats:sup>+<\/jats:sup> lymphocytes, reactive lymphoid nodules, and decreased or normal granulocyte precursor count with left-shifted maturation. In three-color flow cytometry (FCM), T-LGL leukemia cells demonstrated CD2, CD3, and CD8 expression as well as a combination of CD16, CD56, or CD57. Abnormalities of other T-cell antigen expressions (especially CD5, CD7, and CD43) were also detected. In patients with polyclonal T-LGL lymphocytosis, T cells were dispersed in the bone marrow and the expression of pan-T-cell antigens in FCM was normal. Molecular studies revealed <jats:italic>TCRB<\/jats:italic> and <jats:italic>TCRG<\/jats:italic> gene rearrangements in 13 patients and <jats:italic>TCRB<\/jats:italic>, <jats:italic>TCRG<\/jats:italic>, and <jats:italic>TCRD<\/jats:italic> in 4 patients. The most frequently rearranged regions of variable genes were V<jats:sub>\u03b2<\/jats:sub>-J<jats:sub>\u03b21<\/jats:sub>, J<jats:sub>\u03b22<\/jats:sub> and V<jats:sub>\u03b3<\/jats:sub> If V<jats:sub>\u03b310<\/jats:sub>-J<jats:sub>\u03b3<\/jats:sub>. Moreover, in 4 patients, additional rearrangements of <jats:italic>IG<\/jats:italic> kappa and lambda variable genes of B cells were also observed.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Conclusion<\/jats:title>\n                        <jats:p>RA and neutropenia patients represented a continuous spectrum of T-LGL proliferations, although monoclonal expansions were most frequently observed. The histopathological pattern and immunophenotype of bone marrow infiltration as well as molecular characteristics were similar in T-LGL leukemia patients with and without arthritis.<\/jats:p>\n                     <\/jats:sec>","DOI":"10.1186\/ar2424","type":"journal-article","created":{"date-parts":[[2008,5,13]],"date-time":"2008-05-13T06:13:52Z","timestamp":1210659232000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Characteristics of T-cell large granular lymphocyte proliferations associated with neutropenia and inflammatory arthropathy"],"prefix":"10.1186","volume":"10","author":[{"given":"Monika","family":"Prochorec-Sobieszek","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grzegorz","family":"Rymkiewicz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hanna","family":"Makuch-\u0141asica","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miros\u0142aw","family":"Majewski","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katarzyna","family":"Michalak","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Rupi\u0144ski","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Krzysztof","family":"Warzocha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Renata","family":"Maryniak","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2008,5,12]]},"reference":[{"key":"2274_CR1","doi-asserted-by":"publisher","first-page":"357","DOI":"10.7326\/0003-4819-103-3-357","volume":"103","author":"WJ Wallis","year":"1985","unstructured":"Wallis WJ, Loughran TP, Kadin ME, Clark EA, Starkebaum GA: Polyarthritis and neutropenia associated with circulating large granular lymphocytes. 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