Analysis Of Clinical And Laboratory Characteristics With Plasma Cell Leukemia Of 25 Patients

Objective: To investigate the clinical and laboratory characteristics with plasma cell leukaemia(PCL),analysis therapeutic effective and prognosis,in order to raise awareness of the disease.Methods: A total of 25 patients of PCL of The First Hospital Of China Medical University from August 2011 to January 2018 were retrospectively analyzed.Results: The primary plasma cell leukemia in 18 cases,7 cases of secondary plasma cell leukemia;There were 13 males and 12 females,the proportion of male and female was1.08:1,there were 32 to 71 years of age,with a median age at onset of 55.14±4.54 years;fatigue in 18 cases(72.0%),15 cases of weight loss(60.0%),bone pain in 13cases(52.0%),splenomegaly in 11 cases(44.0%),8 cases of lymphadenopathy(32.0%),8cases of anemia(32%),7 cases of fever(28.0%),6 cases of hemorrhage(24%),large liver in3 cases(12.0%);Peripheral blood and bone marrow cell morphology showed a large number of plasma cell infiltration,with abnormal morphology;13 cases of immunophenotype expressed plasma-associated antigen CD38 and CD138,do not express CD10?CD21 and CD3?CD4?CD8?CD25;4 cases were analyzed by chromosome and the result was 2 cases of normal karyotype,2 cases of clonal abnormalities;2 cases of FISH,including 1 cases of 13q-,RB1 gene deletion;14 patients were treated based on bortezomib,the total effective rate was 71.4%,of which 2 acquired CR,2 VGPR and 6PR;7 cases of conventional chemotherapy,3 received PR,2 failed to evaluate the efficacy,and 2 died within 1 month of diagnosis;4 cases were treated with thalidomide alone,Combined dexamethasone or ubenimex,of which 1 was SD,1 failed to evaluate the efficacy and 2 died within 2.5 months of diagnosis;The ORR of 14 patients receiving bortezomib-based chemotherapy was significantly higher than that of 11 patients receiving non-bortezomib(P=0.047);The median survival time of all patients was 10.2months.The median survival time of primary and secondary plasma cell leukemia was16.0 months and 3.1 months(P =0.008).The 3-month survival rates were 77.4% and42.9% respectively;The median survival time was 10.8 months in the bortezomib combined with chemotherapy group,which was longer than 8.7 months in the traditional chemotherapy group and 2.2 months in the thalidomide group(P =0.831).Conclusions: PCL as a highly malignant hematological malignancy,clinical manifestations of diverse,rapid progress,immunophenotypic and cytogenetic heterogeneity,short survival,The median survival time of patients with primary plasma cell leukemia was significantly longer than that of secondary.The new drug bortezomib combined with chemotherapy increased the response rate,the survival period than the traditional chemotherapy and single drug thalidomide extended,providing patients with new hope for treatment.