{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Mahmoud AM"],"funding":["Associazione Italiana per la Ricerca sul Cancro","Ministero dell'Università e della Ricerca","Ministero della Salute","University of Eastern Piedmont Amadeo Avogadro","Ministero dell'Università e della Ricerca","Associazione Italiana Contro le Leucemie Linfomi e Mieloma"],"pagination":["1015"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9954516"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["15(4)"],"pubmed_abstract":["Richter Syndrome (RS) is defined as the development of an aggressive lymphoma in patients with a previous or simultaneous diagnosis of chronic lymphocytic leukemia (CLL). Two pathological variants of RS are recognized: diffuse large B-cell lymphoma (DLBCL)-type and Hodgkin lymphoma (HL)-type RS. Different molecular mechanisms may explain the pathogenesis of DLBCL-type RS, including genetic lesions, modifications of immune regulators, and B cell receptor (BCR) pathway hyperactivation. Limited data are available for HL-type RS, and its development has been reported to be similar to de novo HL. In this review, we focus on the immune-related pathogenesis and immune system dysfunction of RS, which are linked to BCR over-reactivity, altered function of the immune system due to the underlying CLL"],"journal":["Cancers"],"pubmed_title":["Immunological Aspects of Richter Syndrome: From Immune Dysfunction to Immunotherapy."],"pmcid":["PMC9954516"],"funding_grant_id":["AGING project","PRIN 2015ZMRFEA","2022","5 x 1000 No. 21198","project RF-2018-12365790"],"pubmed_authors":["Mahmoud AM","Mouhssine S","Gaidano G"],"additional_accession":[]},"is_claimable":false,"name":"Immunological Aspects of Richter Syndrome: From Immune Dysfunction to Immunotherapy.","description":"Richter Syndrome (RS) is defined as the development of an aggressive lymphoma in patients with a previous or simultaneous diagnosis of chronic lymphocytic leukemia (CLL). Two pathological variants of RS are recognized: diffuse large B-cell lymphoma (DLBCL)-type and Hodgkin lymphoma (HL)-type RS. Different molecular mechanisms may explain the pathogenesis of DLBCL-type RS, including genetic lesions, modifications of immune regulators, and B cell receptor (BCR) pathway hyperactivation. Limited data are available for HL-type RS, and its development has been reported to be similar to de novo HL. In this review, we focus on the immune-related pathogenesis and immune system dysfunction of RS, which are linked to BCR over-reactivity, altered function of the immune system due to the underlying CLL","dates":{"release":"2023-01-01T00:00:00Z","publication":"2023 Feb","modification":"2025-04-25T17:03:22.16Z","creation":"2025-04-25T17:03:22.16Z"},"accession":"S-EPMC9954516","cross_references":{"pubmed":["36831361"],"doi":["10.3390/cancers15041015"]}}