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Blood, 1 September 2002, Vol. 100, No. 5, pp. 1648-1654
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Splenic marginal zone lymphoma: clinical characteristics and
prognostic factors in a series of 60 patients
Jose I. Chacón,
Manuela Mollejo,
Enriqueta Muñoz,
Patricia Algara,
Marisol Mateo,
Luis Lopez,
Jesús Andrade,
Iciar García Carbonero,
Beatriz Martínez,
Miguel A. Piris, and
Miguel A. Cruz
From the Medical Oncology Service, Pathologic Anatomy
Department, Preventive Medicine and Epidemiological Service, and
Genetics Service, Hospital Virgen de la Salud, Toledo, Spain; CNIO
(Centro Nacional de Investigaciones Oncológicas), Madrid,
Spain.
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.
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