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Rapid and complete response to idelalisib in a case of Richter syndrome

Authors Bagacean C, Zdrenghea M, Saad H, Berthou C, Renaudineau Y, Tempescul A

Received 13 September 2018

Accepted for publication 24 January 2019

Published 13 February 2019 Volume 2019:12 Pages 1181—1184


Checked for plagiarism Yes

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Peer reviewers approved by Dr Andrew Yee

Peer reviewer comments 2

Editor who approved publication: Dr William Cho

Cristina Bagacean,1–3 Mihnea Zdrenghea,4 Hussam Saad,1 Christian Berthou,1,2 Yves Renaudineau,3 Adrian Tempescul1,2

1Department of Hematology, Brest University Medical School Hospital, Brest, France; 2U1227 B Lymphocytes and Autoimmunity, University of Brest, INSERM, IBSAM, Brest, France; 3Laboratory of Immunology and Immunotherapy, Brest University Medical School Hospital, Brest, France; 4Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania

Abstract: Richter syndrome (RS) is an aggressive lymphoma arising on the back of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) and is the most common B-cell malignancy in the Western world. In the majority of cases, RS presents an activated B cell (ABC) phenotype of diffuse large B-cell lymphoma (DLBCL). From the therapeutic point of view, selective inhibition of PI3Kδ with idelalisib represents a valuable addition to available treatment options for patients with CLL/SLL, many of whom do not respond to or cannot tolerate chemoimmunotherapy. However, to our knowledge, there have been no prospective studies evaluating idelalisib efficacy in a DLBCL-ABC form of RS. Here, we present a case of a DLBCL-ABC form of RS achieving a complete response at 3 weeks after initiating idelalisib and rituximab therapy for six cycles. This response was maintained during the idelalisib monotherapy, but the patient relapsed rapidly after treatment was withdrawn, because of a grade three immune colitis that developed at 10 months of treatment. This report demonstrates that idelalisib is highly effective in RS and provides an attractive option in this aggressive disease. This agent could meet an unmet need by providing a treatment option with a tolerable safety profile for elderly patients with RS.

Keywords: chronic lymphocytic leukemia, small lymphocytic lymphoma, Richter syndrome, idelalisib

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