{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T04:27:18Z","timestamp":1789964838336,"version":"4.0.1"},"reference-count":32,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2009,12,13]],"date-time":"2009-12-13T00:00:00Z","timestamp":1260662400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2009,12,13]],"date-time":"2009-12-13T00:00:00Z","timestamp":1260662400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematological Oncology"],"published-print":{"date-parts":[[2010,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Richter's syndrome (RS) represents the transformation of chronic lymphocytic leukaemia (CLL) to aggressive lymphoma and is mostly represented by diffuse large B\u2010cell lymphoma (DLBCL), with a post\u2010germinal centre (GC) phenotype, clonally related to the pre\u2010existing CLL. RS has a very poor prognosis and its pathogenetic mechanisms are poorly understood. In order to gain additional hints in RS pathogenesis, we performed a genome\u2010wide DNA profiling study of 13 RS phases and eight matched CLL phases using the Affymetrix Human Mapping 250K NspI SNP arrays. Individual genomic profiles were heterogeneous, with no individual lesions occurring in more than half of the cases. However, several observations suggest that\n                    <jats:italic>MYC<\/jats:italic>\n                    pathway might be involved in RS. The 13q13.3\u2010qter region containing\n                    <jats:italic>MIRHG1 (MIR\u201017\u201092)<\/jats:italic>\n                    , a cluster of microRNA interacting with\n                    <jats:italic>c\u2010MYC<\/jats:italic>\n                    , was acquired at the time of transformation. The 13q gain was coupled with the gain of\n                    <jats:italic>c\u2010MYC<\/jats:italic>\n                    and loss of\n                    <jats:italic>TP53<\/jats:italic>\n                    . Translocation of\n                    <jats:italic>c\u2010MYC<\/jats:italic>\n                    was acquired at transformation in a fraction of cases and this event appeared mutually exclusive with gain of\n                    <jats:italic>MIRHG1<\/jats:italic>\n                    .\n                    <jats:italic>MYCN<\/jats:italic>\n                    , a\n                    <jats:italic>c\u2010MYC<\/jats:italic>\n                    homologue, was also recurrently gained. By comparing RS with 48\n                    <jats:italic>de novo<\/jats:italic>\n                    DLBCL, RS presented a significantly lower prevalence of deletions affecting the\n                    <jats:italic>PRDM1<\/jats:italic>\n                    and\n                    <jats:italic>TNFAIP3<\/jats:italic>\n                    , genes on 6q, known to be associated with a post\u2010GC phenotype. In conclusion, the genomic profile of RS seems to differ from what observed in\n                    <jats:italic>de novo<\/jats:italic>\n                    DLBCL and in other transformed DLBCL. Genomic lesions occurring in RS are heterogeneous suggesting the existence of different RS subsets, possibly due to different transforming mechanisms. A deregulation of MYC pathway might represent one of the main transformation events in the pathogenesis of a subset of RS clonally related to the previous CLL. Copyright \u00a9 2009 John Wiley &amp; Sons, Ltd.\n                  <\/jats:p>","DOI":"10.1002\/hon.932","type":"journal-article","created":{"date-parts":[[2009,12,13]],"date-time":"2009-12-13T21:07:19Z","timestamp":1260738439000},"page":"62-67","source":"Crossref","is-referenced-by-count":45,"title":["Genomic profiling of Richter's syndrome: recurrent lesions and differences with\n                    <i>de novo<\/i>\n                    diffuse large B\u2010cell lymphomas"],"prefix":"10.1002","volume":"28","author":[{"given":"Marta","family":"Scandurra","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Rossi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara","family":"Deambrogi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola MV","family":"Rancoita","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekaterina","family":"Chigrinova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Mian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Cerri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Rasi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Sozzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Forconi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurilio","family":"Ponzoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Santiago M","family":"Moreno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel A","family":"Piris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgio","family":"Inghirami","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Zucca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valter","family":"Gattei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Rinaldi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivo","family":"Kwee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianluca","family":"Gaidano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Bertoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,12,13]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.20773"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/hon.880"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-01-129726"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64256-3"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1159\/000108315"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.12.5413"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404183"},{"key":"e_1_2_1_9_2","first-page":"1405","article-title":"Aberrant somatic hypermutation in transformation of follicular lymphoma and chronic lymphocytic leukemia to diffuse large B\u2010cell lymphoma","volume":"91","author":"Rossi D","year":"2006","journal-title":"Haematologica"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07398.x"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-08-1630"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-08-3266"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2008.399"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07166.x"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V82.8.2289.2289"},{"key":"e_1_2_1_16_2","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1182\/blood.V83.5.1363.1363","article-title":"Molecular genetic demonstration of the diverse evolution of Richter's syndrome (chronic lymphocytic leukemia and subsequent large cell lymphoma)","volume":"83","author":"Matolcsy A","year":"1994","journal-title":"Blood"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1320-5463.2003.01455.x"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1038\/35085588"},{"key":"e_1_2_1_19_2","first-page":"1640","article-title":"The immunological profile of B\u2010cell disorders and proposal of a scoring system for the diagnosis of CLL","volume":"8","author":"Matutes E","year":"1994","journal-title":"Leukemia"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-05-1545"},{"key":"e_1_2_1_21_2","article-title":"Genome wide DNA\u2010profiling of HIV\u2010related B\u2010cell lymphomas","author":"Capello D","year":"2009","journal-title":"Br J Haematol"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2105-10-10"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pcbi.0020041"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0804295105"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20617"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature03552"},{"key":"e_1_2_1_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.12.027"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31804bdaf8"},{"key":"e_1_2_1_29_2","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20052204"},{"key":"e_1_2_1_30_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-07-2950"},{"key":"e_1_2_1_31_2","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-03-4056"},{"key":"e_1_2_1_32_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature07968"},{"key":"e_1_2_1_33_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-02-140616"}],"container-title":["Hematological Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhon.932","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/hon.932","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/hon.932","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T17:15:45Z","timestamp":1772990145000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/hon.932"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,12,13]]},"references-count":32,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2010,6]]}},"alternative-id":["10.1002\/hon.932"],"URL":"https:\/\/doi.org\/10.1002\/hon.932","archive":["Portico"],"relation":{},"ISSN":["0278-0232","1099-1069"],"issn-type":[{"value":"0278-0232","type":"print"},{"value":"1099-1069","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,12,13]]}}}