title: Dual institution experience of nodal marginal zone lymphoma reveals excellent long-term outcomes in the rituximab era.
abstract: Nodal marginal zone lymphoma (NMZL) is a rare non-Hodgkin lymphoma that arises from mature B-cells. We delineate outcomes, prognostic factors and treatment trends among a large cohort of patients with NMZL in the rituximab era. We identified 56 such patients treated at our institutions. The majority presented with advanced stage disease (78·6%). Over a median follow-up of 38·2 months, median progression-free survival (PFS) was 42·4 months and median overall survival (OS) was not reached. Kaplan-Meier estimates of OS at 120 months after diagnosis was 71·9%. High-risk follicular lymphoma international prognostic index (FLIPI) was associated with inferior PFS. Age >60 years and elevated serum lactate dehydrogenase (LDH) were associated with inferior OS. Transformation to diffuse large B-cell lymphoma occurred in 7 patients, 6 of who presented with advanced disease. OS was comparable to our previously reported extranodal MZL cohort. FLIPI score predicted for inferior PFS and OS when both cohorts were analysed together (n = 267). In summary, outcomes in NMZL are favourable with a large majority of patients surviving at 120 months. High risk FLIPI, age >60 years, and elevated serum LDH were associated with inferior outcomes.
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Adam G
authors.initials: AG
authors.lastname: Starr
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Paolo F
authors.initials: PF
authors.lastname: Caimi
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: PingFu
authors.initials: P
authors.lastname: Fu
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Mira R
authors.initials: MR
authors.lastname: Massoud
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Howard
authors.initials: H
authors.lastname: Meyerson
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Eric D
authors.initials: ED
authors.lastname: Hsi
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: David B
authors.initials: DB
authors.lastname: Mansur
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Sheen
authors.initials: S
authors.lastname: Cherian
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Brenda W
authors.initials: BW
authors.lastname: Cooper
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Marcos J G
authors.initials: MJ
authors.lastname: De Lima
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Hillard M
authors.initials: HM
authors.lastname: Lazarus
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Stanton L
authors.initials: SL
authors.lastname: Gerson
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Deepa
authors.initials: D
authors.lastname: Jagadeesh
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Mitchell R
authors.initials: MR
authors.lastname: Smith
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Dean
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Brad L
authors.initials: BL
authors.lastname: Pohlman
authors.affiliation: Cleveland Clinic Foundation, Case Comprehensive Cancer Center, Cleveland, OH, USA.
authors.forename: Brian T
authors.initials: BT
authors.lastname: Hill
authors.affiliation: University Hospitals Case Medical Center/Case Western Reserve University, Cleveland, OH, USA. basem.william@osumc.edu.
authors.forename: Basem M
authors.initials: BM
authors.lastname: William
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D015415:Biomarkers; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D000069283:Rituximab / Q000008:administration & dosage / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D015415:Biomarkers; D000069283:Rituximab
keywords: B-cells; malignant haematology; non-Hodgkin's lymphoma