title: Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients.
abstract: A precise description of clinical features at presentation and analysis of clinical and biologic prognostic factors in splenic marginal zone lymphoma (SMZL) are still lacking. Here we describe the clinical and biologic features of a series of 60 SMZL patients diagnosed after splenectomy. Analysis for overall survival (OS), failure-free survival (FFS), and the probability of obtaining a response was performed using univariate and multivariate tests. The median age of the patient was 63 years (range, 35-84 years). Performance status according to the Eastern Cooperative Oncology Group (ECOG scale) was 0 = 16%, 1 = 58%, and 2 = 25%. Of the 60 patients, 53 (86.6%) were at Ann Arbor stage IV. All 60 patients received splenectomies, 29 of 60 also received chemotherapy, and 2 received spleen radiotherapy. A complete response (CR) was achieved by 38.3% of patients, and a partial response (PR) was achieved by 55%. Mean OS of the series was 103 months (range, 2-164 months); mean FFS was 40 months (range, 3-164 months). At 5 years from diagnosis, 39 patients (65%) were alive. Patients dying from the disease had a relatively aggressive clinical course, with a short survival (17.5 months [range, 2-72 months]). Significant prognostic factors in multivariate analysis were (1) (for OS and FFS) lack of response to therapy (CR versus noncomplete response [nCR]) and involvement of nonhematopoietic sites, and (2) (for the probability of obtaining CR) bone marrow involvement. Chemotherapy did not influence OS or FFS. p53 overexpression predicted a shorter OS in the univariate analysis. These data confirm the relative indolence of this disease, indicating the existence of a subset of more aggressive cases, which should stimulate the search for predictive biologic factors and alternative therapies.
authors.affiliation: Medical Oncology Service, Pathologic Anatomy Department, Hospital Virgen de la Salud, Toledo, Spain.
authors.forename: Jose I
authors.initials: JI
authors.lastname: Chacón
authors.forename: Manuela
authors.initials: M
authors.lastname: Mollejo
authors.forename: Enriqueta
authors.initials: E
authors.lastname: Muñoz
authors.forename: Patricia
authors.initials: P
authors.lastname: Algara
authors.forename: Marisol
authors.initials: M
authors.lastname: Mateo
authors.forename: Luis
authors.initials: L
authors.lastname: Lopez
authors.forename: Jesús
authors.initials: J
authors.lastname: Andrade
authors.forename: Iciar García
authors.initials: IG
authors.lastname: Carbonero
authors.forename: Beatriz
authors.initials: B
authors.lastname: Martínez
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Piris
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Cruz
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D016393:Lymphoma, B-Cell / Q000401:mortality / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D013160:Splenic Neoplasms / Q000401:mortality / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D016019:Survival Analysis