Richter Syndrome

• Transformation of CLL into an aggressive large B-cell lymphoma (seen in 3-10% of cases)
• Inactivation of TP53 and of CDKN2A is a main mechanism in the transformation to Richter syndrome
• Manifests with weight loss, fevers, night sweats, muscle wasting, (i.e., B symptoms) and increasing hepatosplenomegaly and lymphadenopathy.
• Treatment remains challenging and prognosis poor