title: Richter's transformation to diffuse large B-cell lymphoma: a retrospective study reporting clinical data, outcome, and the benefit of adding rituximab to chemotherapy, from the Israeli CLL Study Group.
abstract: Richter's syndrome (RS) is the rare development of an aggressive lymphoid malignancy in a patient with pre-existing chronic lymphocytic leukemia (CLL). Data on RS is sparse and mostly derived from case reports or small series of patients and only a few larger cohorts have been published. The purpose of this large retrospective study was to summarize our national experience with RS in CLL, examine possible risk factors, and analyze relevant demographic, laboratory and clinical parameters, including results of therapy and outcome. We first evaluated data obtained from 119 patients with RS diagnosed during 1971-2010 from 12 medical centers in Israel. The final cohort summarized consisted of 81 patients with RS who developed only diffuse large B-cell lymphoma (DLBCL) after exclusion all cases with insufficient data and those who were not DLBCL. Median overall survival from time of diagnosis of RS was 8 months; after applying the Richter score, patients could be stratified into three prognostic groups, while all other clinical and laboratory parameters evaluated had no prognostic significance. Prior therapy for CLL had no impact on RS survival (P = 0.8) and patients with therapy "naïve" RS and those who had already received chemotherapy prior to developing RS, had the same survival. The addition of rituximab to chemotherapy for RS improved 2 years overall survival from 19% in the chemotherapy alone arm to 42% (P value of 0.001). Although prognosis of patients with RS remains dismal, this retrospective observation provides support for the use of chemo-immunotherapy in DLBCL-RS.
authors.affiliation: Hematology Unit, Bnai-Zion Medical Center, Haifa.
authors.forename: Tamar
authors.initials: T
authors.lastname: Tadmor
authors.forename: Lev
authors.initials: L
authors.lastname: Shvidel
authors.forename: Osnat
authors.initials: O
authors.lastname: Bairey
authors.forename: Neta
authors.initials: N
authors.lastname: Goldschmidt
authors.forename: Rosa
authors.initials: R
authors.lastname: Ruchlemer
authors.forename: Riva
authors.initials: R
authors.lastname: Fineman
authors.forename: Naomi
authors.initials: N
authors.lastname: Rahimi-Levene
authors.forename: Yair
authors.initials: Y
authors.lastname: Herishanu
authors.forename: Mona
authors.initials: M
authors.lastname: Yuklea
authors.forename: Ariela
authors.initials: A
authors.lastname: Arad
authors.forename: Ariel
authors.initials: A
authors.lastname: Aviv
authors.forename: Aaron
authors.initials: A
authors.lastname: Polliack
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007557:Israel / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000188:drug therapy / Q000473:pathology* / Q000628:therapy; D007958:Leukocyte Count; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D012372:ROC Curve; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab; D016879:Salvage Therapy; D012720:Severity of Illness Index; D063189:Symptom Assessment; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab