Richter Syndrome: Clinical Characteristic, Treatment, and Outcome in a Single Center

Context Richter syndrome (RS) is the occurrence of an aggressive lymphoma on a background of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). It has an incidence of 0.5-1.0%/year with overall prevalence of 2-10% in CLL/SLL. Most cases are of diffuse large B-cell lymphoma subtype (DLBCL-RS) while 5-10% are Hodgkin variant RS (HvRS). Prognosis of patients developing RS is poor with reported overall survival of 1-2 years. In this study, we report the clinical characteristic, treatment and outcome of RS in a single center over a 12-year period. Design All patients with biopsy-confirmed high-grade lymphoma with a background of CLL/SLL between 2004-2015 were identified retrospectively from the histology database of The Royal Marsden Hospital. Information was extracted from Electronic Patient Records (EPR). We analyzed CLL/SLL treatments prior to RS, RS treatment, time from CLL/SLL diagnosis to RS, TP53 disruption status (deletion or mutation), and overall survival (OS). OS was defined as the time from RS to all-cause mortality or last clinic visit. Results 41 patients were identified. The median age at RS diagnosis was 68 years (range 37-85 years) with 3:2 male preponderance. 36 cases (88%) were DLBCL-RS while 5 (12%) were HvRS. Among 30 cases with known CLL TP53 status, 40% had TP53 disruption while 60% did not. 29 patients (71%) had received CLL/SLL treatment while 12 (29%) were treatment-naive. The median time from CLL/SLL diagnosis to RS was 5 years (range 0-26 years). Among DLBCL-RS cases, 75% received R-CHOP/CHOP as first-line treatment, 8% had other regimens, and 17% received supportive care. All HvRS patients received ABVD. The median OS for RS was 16 months (range 0-146). Patients who had received ≥2 CLL/SLL treatment lines had an inferior median OS compared to Conclusion Overall survival in our RS cohort was similar to other published reports. DLBCL-RS, TP53 disruption and ≥ 2 lines of previous CLL/SLL treatment lines were associated with a worse outcome.