Abstract: Objective:To investigate the clinical features and treatment of primary plasma cell leukemia (PPCL).Methods: The data of 22cases of PPCL patients that were admitted to the first hospital of China Medical University between 1987 and 2009 were retrospectively analyzed. Results: Of the 22cases, there were13males and 9 females. Patient age ranged from 33to 76with a mean age of 53.3 ± 13.3 years. The major clinical features were as follows: 19cases had pale appearance, 17cases had fatigue, 15cases had ostealgia, 9 cases had fever, 9 cases had bleeding,7 cases had weight loss, 7 cases had hepatomegaly, 11cases had splenomegaly, 7 cases had lymph node enlargement, 6 cases had pleural effusion, 4 cases had ascites, 2 cases had pericardial effusion, 2 cases had pelvic effusion, and 3 cases had bilateral ede-ma in the lower extremities. Laboratory examinations showed that WBC increase (68.2%), anemia (86.4%), thrombocytope nia (86.4%), serum M-protein increase, renal insufficiency, hypoproteinemia, and ESR and serum uric acid increase were common. Bone destruction was common. Clinical remission was difficult to achieve or maintain by classic chemotherapy. Ten patients received classic chemotherapy that resulted in temporary complete remission (CR) in1 patient, partial remis-sion (PR) in 6 patients, and progression in 3 patients. One patient received classic chemotherapy combined with thalido -mide and achieved CR for 7 months; 3 patients were given Bortezomib combined with chemotherapy or thalidomide, and all of them achieved PR; 2 patients were not given chemotherapy due to lung infection, dysfunction of the liver or cardiac in-sufficiency. One survived for 1 month, while the other survived for4 months; 1 patient died before the therapy started; 5 pa-tients abandoned treatment. Conclusion:PPCL has the clinical characteristics of both acute leukemia and multiple myelo-ma, and is more prone to be acute leukemia with a very poor prognosis. Bortezomib plays an important part in the targeted therapy for PPCL, which provides new hope for PPCL patients. Bortezomib combined with chemotherapy and thalidomide may improve the therapeutic effect.