{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T09:10:30Z","timestamp":1764493830407},"reference-count":23,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,10,25]],"date-time":"2015-10-25T00:00:00Z","timestamp":1445731200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Scand J Immunol"],"published-print":{"date-parts":[[2015,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Granulomatous lymphomatosis is an Epstein\u2013Barr virus (<jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>)\u2010driven B cell proliferation associated with an exuberant <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4<jats:sup>+<\/jats:sup> T cell reaction with usually histopathological pictures of angiocentrism. So far, the characteristics of <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4<jats:sup>+<\/jats:sup> T cells in granulomatous lymphomatosis and the mechanism leading to their expansion remain poorly explored. We report a 56\u2010year\u2010old female with a past history of cold agglutinin disease, which was successfully treated with 4 weekly infusions of rituximab. She presented one year later with features of granulomatous lymphomatosis that resulted in severe lung and bone marrow infiltration. We provide evidence that <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4<jats:sup>+<\/jats:sup> T cell expansion was oligoclonal, involved anergic cells and did not result from an <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>\u2010driven stimulation. Rather, it resulted possibly from a high production of interleukin\u201010 by immunoblastic <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>\u2010positive B cells. The outcome was remarkably favourable with rituximab and steroids. Our results suggest that an <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>\u2010driven B cell proliferation should be investigated in patients presenting with a <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4<jats:sup>+<\/jats:sup> T cells alveolitis or other systemic manifestations resulting from a <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4<jats:sup>+<\/jats:sup> T cell expansion. These features should prompt to introduce an immunosuppressive therapy including steroids and rituximab. Our results deserve further investigations to confirm our pathophysiological hypotheses in <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4<jats:sup>+<\/jats:sup> T cell expansions associated with <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>\u2010driven B cell proliferations and to assess whether granulomatous lymphomatosis could result from comparable mechanisms.<\/jats:p>","DOI":"10.1111\/sji.12370","type":"journal-article","created":{"date-parts":[[2015,9,2]],"date-time":"2015-09-02T13:51:01Z","timestamp":1441201861000},"page":"532-538","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Epstein\u2013Barr virus\u2010driven B Cell Proliferation with <scp>CD<\/scp>4<sup>+<\/sup> T Cell Expansion: A Lymphomatoid Granulomatosis\u2010like Disease Related to Hyperinterleukin\u201010 Secretion of Remarkably Favourable Outcome with Rituximab"],"prefix":"10.1111","volume":"82","author":[{"given":"P.","family":"Cervera","sequence":"first","affiliation":[{"name":"Service d'Anatomopathologie AP\u2010HP H\u00f4pital Saint\u2010Antoine Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Guihot","sequence":"additional","affiliation":[{"name":"Laboratory of Immunology AP\u2010HP Groupe Hospitalier Piti\u00e9\u2010Salp\u00e9tri\u00e8re Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Gorochov","sequence":"additional","affiliation":[{"name":"Laboratory of Immunology AP\u2010HP Groupe Hospitalier Piti\u00e9\u2010Salp\u00e9tri\u00e8re Paris France"},{"name":"Sorbonne Universit\u00e9 UPMC Univ Paris 06 Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Lassoued","sequence":"additional","affiliation":[{"name":"Service d'Immunologie UFR de M\u00e9decine Amiens 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