{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T07:12:09Z","timestamp":1767165129213,"version":"build-2238731810"},"reference-count":23,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2008,7,10]],"date-time":"2008-07-10T00:00:00Z","timestamp":1215648000000},"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":[[2008,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    X\u2010linked lymphoproliferative (XLP) syndrome is a rare primary immune\u2010deficiency disorder caused by mutations of the\n                    <jats:italic>SH2D1A<\/jats:italic>\n                    or\n                    <jats:italic>XIAP<\/jats:italic>\n                    genes. Males with the disorder are usually in good health until contracting Epstein\u2013Barr virus (EBV) whereupon the majority of patients die from fulminant infectious mononucleosis, lymphoma or hypogammaglobulinaemia. This report describes a female carrier with an XLP phenotype who was retrospectively identified after her grandson died from the disorder. Subsequent genetic testing identified the patient's mother and affected maternal grandmother as XLP carriers. The family\u2019s medical records were significant. The proband had lymphoma at ages 2 and 8 and made a full recovery following treatment. Both the maternal grandmother and uncle died of non\u2010Hodgkin\u2019s lymphoma. We were concerned that the XLP carrier mother may be predisposed to lymphoma if the normal X chromosome is skewed towards inactivation. The human androgen receptor assay detected random X chromosome inactivation in the carrier mother. EBV was not detected in the lymphoma tissues of the proband and his grandmother, confirming previous findings that EBV is not always associated with lymphoma in XLP. More significantly, our study highlights the importance of identifying XLP in families with a high incidence of lymphoma.\n                  <\/jats:p>","DOI":"10.1111\/j.1365-3083.2008.02128.x","type":"journal-article","created":{"date-parts":[[2008,7,10]],"date-time":"2008-07-10T07:26:52Z","timestamp":1215674812000},"page":"153-158","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Follicular Lymphoma in a X\u2010linked Lymphoproliferative Syndrome Carrier Female"],"prefix":"10.1111","volume":"68","author":[{"given":"S.\u2010T.","family":"Woon","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Ameratunga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Croxson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Taylor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Neas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Edkins","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Browett","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Gane","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Munn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2008,7,10]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(75)92004-8"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/nm1189"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20261"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/26683"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1038\/2424"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.23.13765"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-065X.2003.00023.x"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature05257"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1038\/nri854"},{"key":"e_1_2_6_11_2","first-page":"2964","article-title":"Southern 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