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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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CLINICAL TRIALS AND OBSERVATIONS

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, Emilio Iannitto, for the Intergruppo Italiano Linfomi

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

The Integruppo Italiano Linfomi (IIL) carried out a study to assess the outcomes of splenic marginal zone lymphoma and to identify prognostic factors in 309 patients. The 5-year cause-specific survival (CSS) rate was 76%. In univariate analysis, the parameters predictive of shorter CSS were hemoglobin levels below 12 g/dL (P < .001), albumin levels below 3.5 g/dL (P = .001), International Prognostic Index (IPI) scores of 2 to 3 (P < .001), lactate dehydrogenase (LDH) levels above normal (P < .001), age older than 60 years (P = .01), platelet counts below 100 000/µL (P = .04), HbsAg-positivity (P = .01), and no splenectomy at diagnosis (P = .006). Values that maintained a negative influence on CSS in multivariate analysis were hemoglobin level less than 12 g/dL, LDH level greater than normal, and albumin level less than 3.5 g/dL. Using these 3 variables, we grouped patients into 3 prognostic categories: low-risk group (41%) with no adverse factors, intermediate-risk group (34%) with one adverse factor, and high-risk group (25%) with 2 or 3 adverse factors. The 5-year CSS rate was 88% for the low-risk group, 73% for the intermediate-risk group, and 50% for the high-risk group. The cause-specific mortality rate (x 1000 person-years) was 20 for the low-risk group, 47 for the intermediate-risk group, and 174 for the high-risk group. This latter group accounted for 54% of all lymphoma-related deaths. In conclusion, with the use of readily available factors, this prognostic index may be an effective tool for evaluating the need for treatment and the intensity of therapy in an individual patient.


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