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Primary systemic anaplastic large cell lymphoma (S-ALCL) is a rarely diagnosed disease with highly heterogeneity and variable outcome.Although people have been identified Anaplastic lymphoma kinase (ALK) as an important biomarker related with S-ALCLs unique clinical features and prognosis, the outcomes of some patients are still unpredictable.So, it is important to find out other valuable prognostic factors to help guide the treatment.In this study, we retrospectively analyzed the data of 92 patients with S-ALCL diagnosed between 2001 and 2007 in Lymphoma Lab of Peking University Health Science Centre, investigated their clinical features and several biological factors: the expression of ALK, Ki-67, BCL-2, WTland the long-term survival, Try to find new combinations of prognostic factors for guiding ALCL treatment according to different risk factors.Our results showed that (1) the median age of the 92 patients was 26 (male/female ratio 1.59) and 50% patients is stage Ⅲ/Ⅳ.(2) Of the 80 valuable patients, with a median follow-up of 46.5 months, 3-year and 5-year survival rate was 63% and 60%, respectively.(3) Univariate analysis demonstrated that stage, the expression of ALK and Ki-67 were significant prognostic factors.(4) The expression of WT1 and BCL-2 were correlated with the expression of ALK.But we couldnt find WT1 had prognostic significance.(5) Stage was an independent prognostic factor according to multivariate analysis.In conclusion, clinical stage Ⅲ-Ⅳ, ALK-negative expression and Ki-67-high expression were significantly associated with of treatment failure in S-ALCL.Combining three poor risk factors for prognosis will be more helpful for guidance of the treatment.