title: Splenic marginal zone lymphoma: excellent outcomes in 64 patients treated in the rituximab era.
abstract: OBJECTIVE
  Splenic marginal zone lymphoma (SMZL) is a rare non-Hodgkin lymphoma. We sought to identify prognostic factors and define outcomes in a cohort of 64 patients with SMZL who were treated at two large academic medical centers in North America in the rituximab era.
  
  
  RESULTS
  Over a median follow-up of 37.8 (range 6-167.1) months, Kaplan-Meier estimate of median OS was 156.3 months and median PFS was 52.9 months. On univariate analysis, baseline hemoglobin <12 g/dl was associated with inferior OS (p = 0.045). High-risk FLIPI score was associated with inferior PFS when compared with intermediate/low risk (p = 0.05) and marginally significant with regard to OS (p = 0.056). Splenectomy was not predictive of OS or PFS (p = 0.563 and 0.937, respectively). Transformation to diffuse large B-cell lymphoma occurred in four (6.3%) patients during the observation period. OS was comparable to contemporaneous cohorts of patients with extranodal and nodal marginal lymphomas and FLIPI score was highly predictive for inferior PFS and OS when all three cohorts were analyzed together.
  
  
  CONCLUSIONS
  Outcomes of SMZL, in our series, were excellent, with a median OS of >13 years. Low hemoglobin and high-risk FLIPI were associated with inferior outcomes.
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Adam G
authors.initials: AG
authors.lastname: Starr
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Paolo F
authors.initials: PF
authors.lastname: Caimi
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: PingFu
authors.initials: P
authors.lastname: Fu
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Mira R
authors.initials: MR
authors.lastname: Massoud
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Howard
authors.initials: H
authors.lastname: Meyerson
authors.affiliation: b Cleveland Clinic Foundation , Case Comprehensive Cancer Center , Cleveland , OH , USA.
authors.forename: Eric D
authors.initials: ED
authors.lastname: Hsi
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: David B
authors.initials: DB
authors.lastname: Mansur
authors.affiliation: b Cleveland Clinic Foundation , Case Comprehensive Cancer Center , Cleveland , OH , USA.
authors.forename: Sheen
authors.identifier: http://orcid.org/0000-0002-7210-0997
authors.initials: S
authors.lastname: Cherian
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Brenda W
authors.initials: BW
authors.lastname: Cooper
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Marcos J G
authors.initials: MJG
authors.lastname: De Lima
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Hillard M
authors.initials: HM
authors.lastname: Lazarus
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Stanton L
authors.initials: SL
authors.lastname: Gerson
authors.affiliation: b Cleveland Clinic Foundation , Case Comprehensive Cancer Center , Cleveland , OH , USA.
authors.forename: Deepa
authors.initials: D
authors.lastname: Jagadeesh
authors.affiliation: b Cleveland Clinic Foundation , Case Comprehensive Cancer Center , Cleveland , OH , USA.
authors.forename: Mitchell R
authors.identifier: http://orcid.org/0000-0003-1428-8765
authors.initials: MR
authors.lastname: Smith
authors.affiliation: b Cleveland Clinic Foundation , Case Comprehensive Cancer Center , Cleveland , OH , USA.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Dean
authors.affiliation: b Cleveland Clinic Foundation , Case Comprehensive Cancer Center , Cleveland , OH , USA.
authors.forename: Brad L
authors.initials: BL
authors.lastname: Pohlman
authors.affiliation: b Cleveland Clinic Foundation , Case Comprehensive Cancer Center , Cleveland , OH , USA.
authors.forename: Brian T
authors.initials: BT
authors.lastname: Hill
authors.affiliation: a University Hospitals Case Medical Center/Case Western Reserve University , Cleveland , OH , USA.
authors.forename: Basem M
authors.initials: BM
authors.lastname: William
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015415:Biomarkers; D015331:Cohort Studies; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D016017:Odds Ratio; D011379:Prognosis; D000069283:Rituximab / Q000627:therapeutic use*; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D015415:Biomarkers; D000069283:Rituximab
keywords: Splenic marginal zone lymphoma; chemotherapy; non-Hodgkin's lymphoma; rituximab; splenectomy