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Blood, 15 June 2006, Vol. 107, No. 12, pp. 4643-4649.
Prepublished online as a Blood First Edition Paper on February 21, 2006; DOI 10.1182/blood-2005-11-4659.


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Submitted November 28, 2005
Accepted February 3, 2006

Splenic marginal zone lymphoma: a prognostic model for clinical use

Luca Arcaini*, Mario Lazzarino, Nora Colombo, Sara Burcheri, Emanuela Boveri, Marco Paulli, Enrica Morra, Marcello Gambacorta, Sergio Cortelazzo, Alessandra Tucci, Marco Ungari, Achille Ambrosetti, Fabio Menestrina, Lorella Orsucci, Domenico Novero, Alessandro Pulsoni, Maurizio Frezzato, Gianluca Gaidano, Daniele Vallisa, Viviana Minardi, Claudio Tripodo, Vincenzo Callea, Luca Baldini, Francesco Merli, Massimo Federico, Vito Franco, and Emilio Iannitto

Divisions of Hematology and Pathology, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
Divisions of Hematology and Pathology, Ospedale Niguarda Ca' Granda, Milano, Italy
Division of Hematology, Ospedali Riuniti, Bergamo, Italy
Ospedali Civili, Divisions of Hematology and Pathology, Brescia, Italy
Departments of Clinical and Experimental Medicine and of Pathology, University of Verona, Verona, Italy
Divisions of Hematology and Pathology, Azienda Ospedaliera S. Giovanni Battista, Torino, Italy
Department of Hematology, University "La Sapienza", Roma, Italy
Division of Hematology, Ospedale S. Bortolo, Vicenza, Italy
Division of Hematology, Amedeo Avogadro University of Eastern Piedmont, Novara, Italy
Division of Medicine and Hematology, Ospedale G. da Saliceto, Piacenza, Italy
Divisions of Hematology and Pathology, University of Palermo, Palermo, Italy
Bone Marrow Transplant Unit, Azienda Ospedaliera Bianchi-Melacrino-Morelli, Reggio Calabria, Italy
Department of Hematology, IRCCS Ospedale Maggiore, University of Milano, Milano, Italy
Division of Hematology, Ospedale S.Maria Nuova, Reggio Emilia, Italy
Department of Oncology and Hematology, University of Modena and Reggio Emilia, Modena, Italy

* Corresponding author; email: luca.arcaini{at}unipv.it.

The Integruppo Italiano Linfomi (IIL) carried out a study to assess the outcome of splenic marginal zone lymphoma and to identify prognostic factors. We studied 309 patients: the 5-years cause-specific survival (CSS) was 76%. In univariate analysis the parameters predictive of shorter CSS were hemoglobin < 12 g/dl (p=0.00002), albumin < 3.5 g/dl (p=0.001), IPI 2-3 (p=0.00001), LDH >normal (p=0.00001), age >60 years (p=0.01), platelets < 100,000/ml (p=0.04), HbsAg-positivity (p=0.01), no splenectomy at diagnosis (p=0.006). Hemoglobin < 12 g/dl, LDH >normal, albumin < 3.5 g/dl maintained a negative influence on CSS in multivariate analysis. Using these 3 variables, we identified 3 prognostic categories: low-risk group (41%) with no adverse factors, intermediate-risk group (34%) with one factor, high-risk group (25%) with 2 or 3 factors. The 5-years CSS was 88% for the low-risk, 73% for the intermediate-risk, and 50% for the high-risk group. The cause-specific mortality rate (x1000 p-yrs) was 20 for the low-risk, 47 for the intermediate-risk, and 174 for the high-risk group. This latter group accounted for 54% of all lymphoma-related deaths. In conclusion, using readily available factors, this prognostic index may be a useful tool for evaluating the need for treatment as well as the intensity of therapy in an individual patient.


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