| Primary bone lymphoma(PBL)is a rare disease.There has been paucity of studies addressing its therapy and prognosis.In addition,due to its relatively long survival period,the time of the larger data(> 50 cases)for the PBL reported span of more than 10 years.During this period,the program of lymphoma and bone malignant tumor 's pathology clinical diagnosis,staging and treatment has changed.So far,there was not a detailed description about its characteristics of causes,clinical-pathogenesis and epidemiology,but also did not develop a standard treatment program or guide.Comprehensive treatment has been widely recognized including radiotherapy,chemotherapy,surgery and targeted therapy.But the specific outcome of whether a particular treatment is a prognostic factor for PBL patients is inconclusive.Especially whether the rituximab is able to benefit patients,has been a controversial topic.ObjectiveTo assess the clinical characteristics,outcome,and prognostic factors of PBL and review the literature.Materials and methodsWe retrospectively analyzed 45 PBL patients(aged 3 to 86 years,mean age 45 years,male 21 and female 24),who initially treated in the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2016?21 patients underwent comprehensive treatment(including radiotherapy,chemotherapy,surgery),16 patients chemotherapy alone,1 patient radiotherapy alone,5 patients surgery alone,2 patients no treatment.All data were statistically analyzed by SPSS Statistics for Windows(Version 22.0.Armonk,NY: IBM Corp.).The Kaplan-Meier method was used for survival analysis,and the Log-rank test to analyze the survival rate between the two groups.Variables achieving significant level of P < 0.05 were entered into the COX proportional hazards regression model to complete multivariable analyses.Independent prognostic factors were determined if they had significant effect in the Cox model(P < 0.05).Results1.In 45 cases of primary bone lymphoma(PBL)patients,pain as first symptoms were 80%.A few patients with B symptoms(33%)and soft tissue invasion(44.4%);11.1% with pathological fracture;93.3%(42 cases)were single.The increasing amplitude of LDH(31.1%)was significantly higher than ALP(8.9%).2.In the 45 patients,there were 24 cases(55.3%)in long bone,the rest were flat and irregular bone.Femur(13,28.9%),maxilla and mandible(6,13.3%),pelvis(5,11.1%),spine(4,8.9%),tibia(4,8.9%),humerus(3,6.7%),multiple(3,6.7%),clavicle(2,4.4%),skull(2,4.4%),ribs(2,4.4%),talus(1 case 2.2%).Pathological types were all Non-Hodgkin's Lymphoma,diffuse large B cell lymphoma(DLBCL)in 29 cases(64.4%),followed by: B lymphoblastic lymphoma(B-LBL)7 cases(15.6%),anaplastic large cell lymphoma(ALCL)4 cases(8.9%),marginal zone lymphoma(MZL)2 cases(4.4%),Burkitt lymphoma 2 cases(4.4%)and T cell lymphoma 1 case(2.2%).3.The median survival time was 37 months(1 ~ 52 months)and the average survival time was 32.93 months.The 1,2,3 and 4 year survival rates were 85.8%,65.1%,52.1% and 31.1% respectively;The overall response rate(ORR)was 80%(CR27.9% + PR58.1%)in 43 patients treated with PBL;3 cases stable and 3 cases progressed.4.The average survival time of DLBCL group and non-DLBCL group was 36.4 months,19.6 months respectively,and the 2-years overall survival(OS)was78.1%,56.3%,P = 0.088.5.The mean survival time of radiation therapy group was 50.5 months and 24 months in non-radiotherapy group.The 2 year overall survival was 100%,50.7% respectively,(P = 0.000).But in the COX multivariate correlation analysis,P = 0.926.6.The average survival time of the combined treatment group and the non-combination group were 42.9 months and 24 months,and their two-year OS was 77% and 57.1%(P=0.003).In the COX multivariate correlation analysis,P = 0.837.7.Median survival time of chemotherapy group was 32.9 months and nonchemotherapy group 40.7 months.The 2-years overall survival was 60.9%,66.7%(P = 0.496).8.The values of P and Exp(B)in COX multivariate analysis of PBL prognosis were: staging(0.008,6.908),B symptom(0.686,1.346),moving(0.236,3.239)Conclusions1.The long tubular bone was the most common site of primary bone lymphoma(PBL)involving,especially the femur;pathological type was mostly NHL,more common was diffuse large B cell lymphoma(DLBCL),pain was the common initial symptom;LDH increased more significantly compared with ALP.PBL has high overall survival and comparatibely good prognosis.2.Radiotherapy and combination therapy could prolong the survival time in patients with PBL,clinical attention should pay attention to the role of radiation therapy in the treatment of PBL patients.3.Although this study showed that the addition of rituximab did not benefit patients,but this conclusion still need a large sample of multi-center clinical studies to confirm.4.Clinical staging was an independent factor that affects the prognosis of patients with PBL.In assessing the prognosis of the disease,the clinical stage should be considered more. |
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