Bruton tyrosine kinase inhibitors in combination with chemoimmunotherapy is an effective treatment for patients with Richter’s syndrome

Richter's syndrome (RS) is characterized by transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) into an aggressive lymphoma. While significant treatment advances have been made in the management of CLL/SLL, current front-line management of RS is consistent with chemoimmunotherapy (CIT), a therapeutic approach adopted from the management of de-novo diffuse large B-cell lymphoma (DLBCL). In de-novo DLBCL, CIT alone can potentially cure a significant percentage of patients; however in RS, outcomes with CIT alone are particularly poor. Therefore there is a significant unmet need to improve the current standard of care for the management of RS. We present a case of a patient with newly diagnosed RS who was treated with a novel upfront combination of CIT and a Bruton tyrosine kinase inhibitor, resulting in excellent long-term outcomes. We also present accompanying literature review highlighting other anecdotal cases reported with similar therapeutic approach and discuss emerging treatment strategies in the management of RS.