Diagnosis and management of Richter's syndrome in 2017

Hélène Vellemans, Stéphane Lepretre Published in the journal : June 2018 Category : Hematology/Oncology

Summary :

Richter’s syndrome is a rare syndrome characterized by the development of aggressive lymphomas in patients with concomitant chronic lymphocytic leukemia. Known for several years, this syndrome is still very heterogeneous and represents a real clinical challenge, given its aggressive nature and very limited prognosis. In the era of targeted therapies and due to the increasingly-thorough characterization of the genetic abnormalities of this condition, there is an unmet need for new molecules in order to better manage patients suffering from this syndrome. This article is a brief literature review as regards diagnosis and management of Richter's syndrome in 2017.

Key Words

Chronic lymphocytic leukemia, Richter’s syndrome, Richter’s transformation, Diffuse large B Cell lymphoma (DLBCL), treatment

What is already known about the topic?

Richter’s syndrome is a rare disease that results from the transformation of chronic lymphocytic leukemia, or of low-grade lymphoma, into an aggressive lymphoma. Recently, there is has been no clear therapeutic progress made in this pathological domain; in the near future, however, the advent of new molecules could significantly change this syndrome’s management.

What does this article bring up for us?

This article constitutes a literature review on the epidemiology, diagnostic criteria, and current management of Richter’s syndrome that more particularly focuses on its transformation into diffuse large B-cell lymphoma. It also reviews the molecules currently under study, and potential future treatments to improve the management of this syndrome.