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Review
. 2019 Jan 8;7(4):IJH09.
doi: 10.2217/ijh-2018-0010. eCollection 2018 Dec.

Current trends in the management of Richter's syndrome

John N Allan  1 , Richard R Furman  1
Affiliations
Review

Current trends in the management of Richter's syndrome

John N Allan et al. Int J Hematol Oncol. .

Abstract

Richter's syndrome (RS) is a life-threatening complication of chronic lymphocytic leukemia (CLL). While previous research has increased our knowledge on the distinct evolutionary patterns of RS and provided a deeper understanding of the risk factors and molecular events predisposing to transformation, there remain few targetable aberrations and treatment is largely ineffective. The ability to obtain deeper remissions, without selecting for deletion 17p, by using novel B-cell receptor (BCR) antagonists and bcl2 inhibition might lead to a decrease in the incidence of RS, but these agents have done little to significantly change outcomes when incorporated into treatment regimens for RS. In this review we highlight the current landscape of molecular lesions specific to RS, review the data on historical treatment options, and look to the horizon for potential opportunities in the future.

Keywords: Richter's syndrome; Richter's transformation; management; novel agents.

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Conflict of interest statement

Financial & competing interests disclosure JN Allan is a member of the Advisory Board for Pharmacyclics, Acerta, Abbvie, Sunesis, Verastem, Genentech and Bayer. RR Furman is a member of the Advisory Board for Pharmacyclics, Acerta, Abbvie, Sunesis, Verastem, Genentech and Janssen. Consultancy: Acerta, Verastem, TG Therapeutics and Janssen. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. No writing assistance was utilized in the production of this manuscript.

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