Richter transformation of chronic lymphocytic leukaemia: a British Society for Haematology Good Practice Paper.

RT occurs in 2-10% of CLL patients, usually during the disease course rather than at presentation, representing a transformation rate of 0-5-1% per CLL patient per year.2-5 RT should be suspected when a CLL patient develops one or more new "B symptoms", asymmetric, rapidly progressive lymphadenopathy, or a sudden lactate dehydrogenase (LDH) rise. Treatment approach Patients with RT commonly present in the context of pre-treated CLL and immunosuppression, and given the typical demographics of the CLL population, patients are often older with co-existing comorbidities.22 Treatment has historically involved multi-agent cytotoxic chemotherapy, more recently in combination with an anti-CD20 monoclonal antibody. Patients with I TP53 i aberrations or those who develop RT having previously received CLL-directed treatment have a poor outcome with R-CHOP alone, although this remains the standard of care and provides at least initial disease control for most patients. Diffuse large B-cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients. [Extracted from the article]