A single-institution retrospective cohort study of first-line R-EPOCH chemoimmunotherapy for Richter syndrome demonstrating complex chronic lymphocytic leukaemia karyotype as an adverse prognostic factor

作者：Rogers Kerry A; Huang Ying; Ruppert Amy S; Salem Galena; Stephens Deborah M; Heerema Nyla A; Andritsos Leslie A; Awan Farrukh T; Byrd John C; Flynn Joseph M; Maddocks Kami J; Jones Jeffrey A

来源：British Journal of Haematology, 2018, 180(2): 259-266.

DOI：10.1111/bjh.15035

• Richter syndrome
• chronic lymphocytic leukaemia (CLL)
• chemotherapy

Richter Syndrome, an aggressive lymphoma occurring in patients with chronic lymphocytic leukaemia (CLL), has a generally poor prognosis and anthracycline-based chemoimmunotherapy regimens designed to treat de novo diffuse large B-cell lymphoma achieve modest clinical benefit. REPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) has demonstrated greater activity against aggressive B-cell histologies but has not been studied in Richter Syndrome. We conducted a retrospective cohort study of 46 Richter Syndrome patients treated with first-line R-EPOCH at our institution between 1 January 2006 and 31 May 2014. The median progression-free survival (PFS) was 3.5 months [95% confidence interval (CI): 2.0-7.6] and median overall survival (OS) was 5.9 months (95% CI: 3.2-10.3). Toxicity was high and 30% of patients died without progression or response. Patients with a complex CLL karyotype had significantly shorter PFS and OS (P = 0.005 and P = 0.002, respectively). Multivariable analysis identified complex CLL karyotype as the most significant predictor of decreased survival [Hazard ratio (HR) 2.72, 95% CI: 1.14-6.52, P = 0.025], adjusting for number of prior CLL treatments (P = 0.036). Richter Syndrome patients with complex CLL karyotype experience poor survival with R-EPOCH treatment and novel approaches are needed for these patients. In contrast, survival of patients without a complex CLL karyotype was similar to patients with de novo diffuse large B-cell lymphoma.

• 出版日期2018-1