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

摘要

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 significant proportion of patients, constituting almost 20% of SMZL, display a more aggressive clinical course with compromised survival [4]. Histologic transformation to diffuse 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 extra-hilar lymphadenopathy (outside the splenic and hepatic hila) [13].