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Validation of the simplified prognostic score for splenic marginal zone lymphoma of the Splenic Marginal Zone Lymphoma Working Group

Splenic marginal zone lymphoma (SMZL) is a low grade B-cell lymphoma usually characterized by an indolent clinical course and long survival (median 10 years) [1 – 3]. However, a signifi cant proportion of patients, constituting almost 20% of SMZL, display a more aggressive clinical course with compromised survival [4]. Histologic transformation to diff use large B-cell lymphoma is seen in fewer than 10% of patients and is associated with an adverse outcome, since survival after transformation is usually less than 2 years [4 – 6]. Several clinical and biological factors have been proposed for identifying prognostic subgroups in this lymphoma entity, but the results were not reproducible [4 – 12]. Recently, the SMZL Working Group (SMZLWG) proposed a prognostic index based on the combination of four factors: hemoglobin level, platelet count, elevated lactate dehydrogenase (LDH) and the presence of extrahilar lymphadenopathy (outside the splenic and hepatic hila) [13]. Hemoglobin level and platelet count were used as continuous variables to provide the best fi t, which resulted in the following prognostic index: