Molecular Subtypes and the Role of TP53 in Diffuse Large B-Cell Lymphoma and Richter Syndrome.

Autor: Negara I; Molecular Hematology Unit, International Centre for Genetic Engineering and Biotechnology, 34149 Trieste, Italy.; Department of Internal Medicine, Hematology, 'Nicolae Testemitanu' State University of Medicine and Pharmacy, 2004 Chisinau, Moldova., Tomuleasa C; Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy, 400347 Cluj-Napoca, Romania., Buruiana S; Department of Internal Medicine, Hematology, 'Nicolae Testemitanu' State University of Medicine and Pharmacy, 2004 Chisinau, Moldova., Efremov DG; Molecular Hematology Unit, International Centre for Genetic Engineering and Biotechnology, 34149 Trieste, Italy.; Macedonian Academy of Sciences and Arts, 1000 Skopje, North Macedonia.
Jazyk: angličtina
Zdroj: Cancers [Cancers (Basel)] 2024 Jun 07; Vol. 16 (12). Date of Electronic Publication: 2024 Jun 07.
DOI: 10.3390/cancers16122170
Abstrakt: Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy and a heterogeneous entity comprised of several biologically distinct subtypes. Recently, novel genetic classifications of DLBCL have been resolved based on common mutational patterns indicative of distinct pathways of transformation. However, the complicated and costly nature of the novel classifiers has precluded their inclusion into routine practice. In view of this, the status of the TP53 gene, which is mutated or deleted in 20-30% of the cases, has emerged as an important prognostic factor for DLBCL patients, setting itself apart from other predictors. TP53 genetic lesions are particularly enriched in a genetic subtype of DLBCL that shares genomic features with Richter Syndrome, highlighting the possibility of a subset of DLBCL arising from the transformation of an occult chronic lymphocytic leukemia-like malignancy, such as monoclonal B-cell lymphocytosis. Patients with TP53-mutated DLBCL, including those with Richter Syndrome, have a particularly poor prognosis and display inferior responses to standard chemoimmunotherapy regimens. The data presented in this manuscript argue for the need for improved and more practical risk-stratification models for patients with DLBCL and show the potential for the use of TP53 mutational status for prognostication and, in prospect, treatment stratification in DLBCL.
Databáze: MEDLINE
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