Richter Syndrome: Diagnostic and Therapeutic Management

xlomafota13 shared this article with you from Inoreader Richter Syndrome: Diagnostic and Therapeutic Management Via Association française pour l'étude du cancer Bull Cancer. 2021 Apr 22:S0007-4551(21)00120-X. doi: 10.1016/j.bulcan.2021.01.014. Online ahead of print. ABSTRACT Richter syndrome (RS) is defined as the occurrence of an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL) and rarely Hodgkin lymphoma (HL), in a patient with prior or concomitant chronic lymphocytic leukemia (CLL). RS is estimated to occur in 0.5-1 % per year and is associated with adverse outcome. In the vast majority of patients (80 %), RS is clonally related to the prior CLL. Those with unrelated RS appear to have better outcome. The therapeutic approach is based on those of de novo DLBCL or HL. However, even with modern immunochemotherapy regimens, response rate remains low. In eligible patients with related RS, a consolidation by autologous or allogeneic stem-cell transplantation must be proposed. Combinations including therapies targeting BCR or BCL2 and effective in CLL are currently being ev aluated in RS. Novels immunotherapies could be promising approaches based on preliminary results. PMID:33896586 | DOI:10.1016/j.bulcan.2021.01.014 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.