| Objective:To investigate the clinical characteristics,treatment scheme,curative effect,response and prognosis of patients with plasma cell leukemia(PCL)in order to improve the understanding of the disease.Methods:The clinical data of 27 patients with PCL treated in the Department of Hematology of the second hospital of Shanxi Medical University from December 2010 to August2019 were collected,including gender,age of onset,first clinical manifestation,laboratory examination,treatment scheme and curative effect.Their clinical characteristics were summarized.If they had a previous history of multiple myeloma(MM),they were diagnosed as secondary plasma cell leukemia(s PCL)group,and if they had no previous history of MM,they were primary plasma cell leukemia(p PCL)group,The differences of clinical characteristics between the two groups were compared;According to the treatment plan,they were divided into traditional chemotherapy group and new drug treatment group.The therapeutic effect was evaluated and the survival was analyzed.Results:Among the 27 patients with PCL,there were 8 cases of ppcl1 and 9 cases of spcl9.The male to female ratio was 1.7:1.The median age was 62 years old.The first manifestations were fatigue,bleeding,fever,splenomegaly and bone pain.A large number of plasma cell infiltration was seen in the morphological examination of peripheral blood and bone marrow cells.13 cases were detected by immunotyping,all expressed CD38,CD138,8 cases were detected by chromosome karyotype,3 cases were normal and 5 cases showed clonal abnormal changes;Fish was performed in 12 patients,of which 8 were abnormal;In 17 cases of bortezomib based regimen,the total effective rate was 52.9%,which was higher than that in the non bortezomib group,but there was no significant difference between the two groups(P=0.242);The median OS of all patients was 7.379 months,the median PFS was 3.5 months,and the median OS of patients with primary leukemia and secondary plasma cell leukemia was 8.17 vs 2.42 months,the difference was statistically significant(P=0.031).Conclusion:Plasma cell leukemia has strong invasiveness and diverse clinical manifestations.The median survival time of primary plasma cell leukemia is longer than that of secondary plasma cell leukemia and is not sensitive to traditional chemotherapy.However,bortezomib based chemotherapy scheme improves the effective rate and prolongs the survival time of patients.Although the comparison of the two schemes in this study is not statistically significant,it has important clinical significance. |