Abstract: Objective：To analyze clinical features of systemic anaplastic large cell lymphoma (ALCL), and to explore the influence of clinical and pathological characteristics, especially anaplastic lymphoma kinase (ALK) expression status on the prognosis of ALCL patients. Methods：Totally, 43 patients were enrolled and their clinical and pathological characteristics, treatment and survival data were collected. Results：The clinical characteristics, treatment regimens, progress free survival (PFS), and overall survival (OS) had no statistical difference between the ALK positive and ALK negative groups. The univariate analysis indicated that patients with hemophagocytic lymphohistiocytosis (HLH) was a prognostic factor for both PFS and OS in ALCL patients (P＜0.05); and multivariate analysis indicated that HLH was an independent prognostic factor for PFS in those patients (P＜0.05). Conclusions：No statistical difference in PFS or OS was observed between the ALK positive and ALK negative groups of ALCL patients; HLH comorbidity is a prognostic factor affecting the survival of patients.