{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T20:44:11Z","timestamp":1776458651383,"version":"3.51.2"},"reference-count":30,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,5,3]],"date-time":"2024-05-03T00:00:00Z","timestamp":1714694400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Richter transformation refers to the progression of an initially slow-growing small lymphocytic lymphoma\/chronic lymphocytic leukemia (SLL\/CLL) into an aggressive lymphoma, typically diffuse large B-cell lymphoma (DLBCL) or Hodgkin lymphoma.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>The patient presented with a rapid onset of localized cervical swelling, accompanied by monoclonal B-cell lymphocytosis displaying a CLL immunophenotype. The histopathological analysis identified a Burkitt lymphoma (BL) located in the submandibular gland and adjacent lymph node. The patient\u2019s bone marrow displayed a minor infiltration of monoclonal B-cells with a CLL immunophenotype (&amp;lt; 10%). Molecular analysis demonstrated the presence of the same monoclonal rearrangement in the framework region (FR3 region) of the immunoglobulin heavy chain (<jats:italic>IGH<\/jats:italic>) locus. High-throughput sequencing of the immunoglobulin heavy and light chains also confirmed the presence of the same rearrangement in SLL\/CLL and in the Burkitt lymphoma sample, but also highlighted the presence of a second rearrangement in the Burkitt lymphoma cells, not shared with the SLL\/CLL cells in the bone marrow. The patient was treated with DA-EPOCH-R, which lead to a complete metabolic response.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This report provides an exceptionally rare description of a CLL-type monoclonal B-cell lymphocytosis transforming into a very aggressive Burkitt lymphoma in a treatment na\u00efve patient.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fonc.2024.1296238","type":"journal-article","created":{"date-parts":[[2024,5,3]],"date-time":"2024-05-03T04:32:06Z","timestamp":1714710726000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Case report: \u2018Atypical Richter transformation from CLL-type monoclonal B-cell lymphocytosis into Burkitt lymphoma in a treatment na\u00efve patient\u2019"],"prefix":"10.3389","volume":"14","author":[{"given":"Anna\u00efse J.","family":"Jauch","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilaria","family":"Alborelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Reusser","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Albert","family":"Baschong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cyrill","family":"R\u00fctsche","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Bignucolo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jakob","family":"Passweg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Dirnhofer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fatime","family":"Krasniqi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,5,3]]},"reference":[{"key":"B1","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Swerdlow","year":"2017"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12458","article-title":"Diffuse large B-cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients","volume":"162","author":"Parikh","year":"2013","journal-title":"Br J Haematol"},{"key":"B3","first-page":"185","article-title":"Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia","volume":"4","author":"Richter","year":"1928","journal-title":"Am J Pathol"},{"key":"B4","article-title":"Leucemie Lymphoide Chronique secondairement associ\u00e9e \u00e0 une R\u00e9ticulopathie Maligne, Syndrome de Richter","volume":"4","author":"Lortholary","year":"1964","journal-title":"Nouv Rev Fr Hematol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24473","article-title":"Histologic transformation of chronic lymphocytic leukemia\/small lymphocytic lymphoma","volume":"91","author":"Agbay","year":"2016","journal-title":"Am J Hematol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-023-03488-8","article-title":"Correction to: Chronic lymphocytic leukaemia\/small lymphocytic lymphoma and mantle cell lymphoma: from early lesions to transformation","volume":"482","author":"Sander","year":"2023","journal-title":"Virchows Arch"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-09-302174","article-title":"The genetics of Richter syndrome reveals disease heterogeneity and predicts survival after transformation","volume":"117","author":"Rossi","year":"2011","journal-title":"Blood"},{"key":"B8","doi-asserted-by":"publisher","first-page":"2272","DOI":"10.1182\/blood.2021010664","article-title":"Rapidly deteriorating B-CLL at presentation: clonally related Burkitt leukemia as an atypical Richter transformation","volume":"137","author":"Chatzidimitriou","year":"2021","journal-title":"Blood"},{"key":"B9","article-title":"urkitt's type acute lymphoblastic transformation associated with t(8;14) in a case of B cell chronic lymphocytic leukemia","volume":"11","author":"Asou","year":"1997","journal-title":"Leukemia"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1080\/10428194.2016.1256482","article-title":"EBV-positive Richter's syndrome with laboratory features of Burkitt's lymphoma, in Ibrutinib-treated chronic lymphocytic leukemia","volume":"58","author":"Hussaini","year":"2017","journal-title":"Leuk Lymphoma"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1002\/(ISSN)1097-0142","article-title":"Hypercalcemia may indicate Richter's syndrome: report of four cases and review","volume":"79","author":"Beaudreuil","year":"1997","journal-title":"Cancer"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2005.05.0187","article-title":"Clinical outcomes and prognostic factors in patients with richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation","volume":"24","author":"Tsimberidou","year":"2006","journal-title":"J Clin Oncol"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-03-489518","article-title":"Two main genetic pathways lead to the transformation of chronic lymphocytic leukemia to Richter syndrome","volume":"122","author":"Chigrinova","year":"2013","journal-title":"Blood"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-020-0797-x","article-title":"Richter transformation in chronic lymphocytic leukemia (CLL)-a pooled analysis of German CLL Study Group (GCLLSG) front line treatment trials","volume":"35","author":"Al-Sawaf","year":"2021","journal-title":"Leukemia"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2025746","article-title":"Burkitt's lymphoma","volume":"387","author":"Roschewski","year":"2022","journal-title":"N Engl J Med"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-02-585059","article-title":"Monoclonal B-cell lymphocytosis and early-stage chronic lymphocytic leukemia: diagnosis, natural history, and risk stratification","volume":"126","author":"Strati","year":"2015","journal-title":"Blood"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1002\/cyto.a.24507","article-title":"Comprehensive phenotyping of human peripheral blood B lymphocytes in healthy conditions","volume":"101","author":"Carsetti","year":"2022","journal-title":"Cytometry A"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01620-2","article-title":"The 5th edition of the world health organization classification of haematolymphoid tumours: lymphoid neoplasms","volume":"36","author":"Alaggio","year":"2022","journal-title":"Leukemia"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2009.065227","article-title":"Aberrations of the MYC gene in unselected cases of diffuse large B-cell lymphoma are rare and unpredictable by morphological or immunohistochemical assessment","volume":"62","author":"Obermann","year":"2009","journal-title":"J Clin Pathol"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63055-6","article-title":"Simultaneous evaluation of T- and B-cell clonality, t(11;14) and t(14;18), in a single reaction by a four-color multiplex polymerase chain reaction assay and automated high-resolution fragment analysis: a method for the rapid molecular diagnosis of lymphoproliferative disorders applicable to fresh frozen and formalin-fixed, paraffin-embedded tissues, blood, and bone marrow aspirates","volume":"159","author":"Meier","year":"2001","journal-title":"Am J Pathol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-10-1271","article-title":"Clonal relationship of classical hodgkin lymphoma and its recurrences","volume":"17","author":"Obermann","year":"2011","journal-title":"Clin Cancer Res"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2016000794","article-title":"High-throughput sequencing of the B-cell receptor in African Burkitt lymphoma reveals clues to pathogenesis","volume":"1","author":"Lombardo","year":"2017","journal-title":"Blood Adv"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/aqv011","article-title":"Clonality analysis of immunoglobulin gene rearrangement by next-generation sequencing in endemic burkitt lymphoma suggests antigen drive activation of BCR as opposed to sporadic burkitt lymphoma","volume":"145","author":"Amato","year":"2016","journal-title":"Am J Clin Pathol"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-03-012948","article-title":"Over 20% of patients with chronic lymphocytic leukemia carry stereotyped receptors: Pathogenetic implications and clinical correlations","volume":"109","author":"Stamatopoulos","year":"2007","journal-title":"Blood"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V78.9.2372.2372","article-title":"Variable region gene analysis of pathologic human autoantibodies to the related i and I red blood cell antigens","volume":"78","author":"Silberstein","year":"1991","journal-title":"Blood"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.146.12.4385","article-title":"Nucleotide sequence analysis of the V regions of two IgM cold agglutinins. Evidence that the VH4-21 gene segment is responsible for the major cross-reactive idiotype","volume":"146","author":"Pascual","year":"1991","journal-title":"J Immunol"},{"key":"B27","doi-asserted-by":"publisher","first-page":"1991","DOI":"10.1084\/jem.20160201","article-title":"Self-reactive VH4-34-expressing IgG B cells recognize commensal bacteria","volume":"214","author":"Schickel","year":"2017","journal-title":"J Exp Med"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020007039","article-title":"Higher-order connections between stereotyped subsets: implications for improved patient classification in CLL","volume":"137","author":"Agathangelidis","year":"2021","journal-title":"Blood"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-03-343434","article-title":"Is there a role for antigen selection in mantle cell lymphoma? Immunogenetic support from a series of 807 cases","volume":"118","author":"Hadzidimitriou","year":"2011","journal-title":"Blood"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2010.025437","article-title":"Stereotyped patterns of B-cell receptor in splenic marginal zone lymphoma","volume":"95","author":"Zibellini","year":"2010","journal-title":"Haematologica"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2024.1296238\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,3]],"date-time":"2024-05-03T04:32:11Z","timestamp":1714710731000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2024.1296238\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,3]]},"references-count":30,"alternative-id":["10.3389\/fonc.2024.1296238"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2024.1296238","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,3]]},"article-number":"1296238"}}