title: Clinical features of patients with nodal marginal zone lymphoma compared to follicular lymphoma: similar presentation, but differences in prognostic factors and rate of transformation.
abstract: Nodal marginal zone lymphoma (NMZL) is a rare type of B-cell non-Hodgkin lymphoma. This study assessed the clinical features of 56 patients with NMZL in comparison to 46 patients with follicular lymphoma (FL). Patients with NMZL and FL had a largely similar clinical presentation, but patients with FL had a higher disease stage at presentation, more frequent abdominal lymphadenopathy and bone marrow involvement, and showed more common transformation into diffuse large B-cell lymphoma (DLBCL) during the course of disease. Overall survival and event-free survival were similar for patients with NMZL and FL, but factors associated with worse prognosis differed between the two groups. Transformation into DLBCL was associated with a significantly poorer outcome in both groups, but the phenotypes were different: DLBCL arising in FL was mainly of germinal center B-cell phenotype, whereas DLBCL arising in NMZL was mainly of non-germinal center B-cell phenotype.
authors.affiliation: a Department of Pathology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Michiel
authors.initials: M
authors.lastname: van den Brand
authors.affiliation: b Department of Hematology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Walter J F M
authors.initials: WJ
authors.lastname: van der Velden
authors.affiliation: a Department of Pathology , Radboud University Medical Center , Nijmegen , The Netherlands ;|b Department of Hematology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Illja J
authors.initials: IJ
authors.lastname: Diets
authors.affiliation: a Department of Pathology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Geneviève I C G
authors.initials: GI
authors.lastname: Ector
authors.affiliation: c Department for Health Evidence , Radboud University Medical Center , Nijmegen , The Netherlands.
authors.forename: Anton F J
authors.initials: AF
authors.lastname: de Haan
authors.affiliation: b Department of Hematology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Wendy B C
authors.initials: WB
authors.lastname: Stevens
authors.affiliation: a Department of Pathology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Konnie M
authors.initials: KM
authors.lastname: Hebeda
authors.affiliation: a Department of Pathology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Patricia J T A
authors.initials: PJ
authors.lastname: Groenen
authors.affiliation: a Department of Pathology , Radboud University Medical Center , Nijmegen , The Netherlands ;
authors.forename: Han J M
authors.initials: HJ
authors.lastname: van Krieken
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002471:Cell Transformation, Neoplastic; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000401:mortality*; D008224:Lymphoma, Follicular / Q000175:diagnosis* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D010641:Phenotype; D011379:Prognosis; D016019:Survival Analysis; D063189:Symptom Assessment; D055815:Young Adult
keywords: clinical presentation; follicular lymphoma; nodal marginal zone lymphoma; prognosis; transformation