title: Outcomes in splenic marginal zone lymphoma: analysis of 107 patients treated in British Columbia.
abstract: Splenic marginal zone lymphoma (SMZL) accounts for less than 2% of all non-Hodgkin lymphomas. We identified 107 cases diagnosed with SMZL between 1985 and 2012 from the British Columbia Cancer Agency Centre for Lymphoid Cancer and Lymphoma Pathology Databases. Patient characteristics were: median age 67 years (range 30-88), male 40%, stage IV 98%, splenomegaly 93%, bone marrow involvement 96%, peripheral blood involvement 87%. As initial treatment, 52 underwent splenectomy (10 with chemotherapy), 38 chemotherapy alone (21 chemoimmunotherapy containing rituximab, 1 rituximab alone), two antivirals for hepatitis C, and 15 were only observed. The 10-year overall survival for first-line splenectomy versus chemotherapy was 61% and 42%, respectively [Hazard Ratio (HR) 0·48, 95% confidence interval (CI) 0·26-0·88, P = 0·017]. The 10-year failure-free survival (FFS) after first-line splenectomy vs chemotherapy was 39% and 14%, respectively (HR 0·48, 95% CI 0·28-0·80, P = 0·004). Among the 38 patients who received first-line chemotherapy, FFS was similar between those receiving rituximab (n = 22) and those who did not (n = 16) (HR 0·64, 95% CI 0·31-1·34, P = 0·238). Fifteen patients transformed to aggressive lymphoma with median time to transformation of 3·5 years (range 6 months to 12 years) and the 10-year transformation rate was 18%. In conclusion, splenectomy remains a reasonable treatment for patients with SMZL.
authors: Department of Medical Oncology, British Columbia Cancer Agency - Vancouver Island Centre, Victoria, BC, Canada.
authors: Katharine H
authors: KH
authors: Xing
authors: Amrit
authors: A
authors: Kahlon
authors: Brian F
authors: BF
authors: Skinnider
authors: Joseph M
authors: JM
authors: Connors
authors: Randy D
authors: RD
authors: Gascoyne
authors: Laurie H
authors: LH
authors: Sehn
authors: Kerry J
authors: KJ
authors: Savage
authors: Graham W
authors: GW
authors: Slack
authors: Tamara N
authors: TN
authors: Shenkier
authors: Richard
authors: R
authors: Klasa
authors: Alina S
authors: AS
authors: Gerrie
authors: Diego
authors: D
authors: Villa
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001955:British Columbia / Q000453:epidemiology; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000401:mortality* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000401:mortality* / Q000628:therapy*; D015996:Survival Rate; D013997:Time Factors
keywords: chemotherapy; rituximab; splenectomy; splenic marginal zone lymphoma; transformation