Abstract： Objective To study clinical characteristics and prognostic factors in systemic anaplastic large cell lymphoma (S-ALCL). Methods Clinical date of 30 systemic ALCL patients from Department Hemotology of Shanxi Cancer Hospital from December 1998 to September 2009 were reviewed. Results The median age of the 30 patients was 36 years old. The male/female ratio was 1.5:1.18 (60.0 %) 18/30 of the patients had B symptoms, 22 (73.3 %) had Ann Arbor stage Ⅲ-Ⅳ, and 18 (60.0 %) had extranodal disease at diagnosis. 14 (46.7 %) had elevated lactate dehydrogenase (LDH) level. The positive rate of ALK was 18 (60.0 %). Compared with ALK negative patients, ALK-positive patients were younger (u = 3.92, P=0.001).ALK expression and LDH level were important prognostic factors by Long-rank test. Conclusion S-ALCL usually occur in younger patients, with good response to chemotherapy and good prognosis. ALK negative and elevated LDH level are correlated to unfavorable prognosis. Combination chemotherapy is the major choice to these patients. High-dose chemotherapy maybe benefit to the patients who have adverse prognostic factors.