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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
Author(s) -
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.
Publication year - 2018
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.15035
Subject(s) - medicine , chemoimmunotherapy , hazard ratio , vincristine , gastroenterology , rituximab , oncology , chronic lymphocytic leukemia , lymphoma , chemotherapy , cyclophosphamide , leukemia , confidence interval
Summary 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. R‐ EPOCH (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.