| Background and PurposeB-cell lymphoma,unclassifiable,with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma(DLBCL/BL) is a rare and spcific type of aggressive lymphoma. DLBCL/BL harbour intermediate morphological,immunohistochemical and genetical features between Burkitt lymphoma(BL) and diffuse large B-cell lymphoma(DLBCL), thus it is difficult to classify clearly in`pathological diagnose in most cases. This disease was named B-cell lymphoma,unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma, in the 2008 World Health Organization(WHO) Classification. In some cases, the phenotype is consistent with BL but the morphology is atypical,which warrants placing them into this category. In other cases, the tumour cells are morphologically consistent with BL but with strong BCL-2 expression. A case of typical BL with an atypical phenotype is also placed into this category. So far, the report of DLBCL/BL is rare because of the low incidence. In this study, we collected and analyzed the clinical and pathological data of 13 cases of DLBCL/BL in order to promoting the cognition of this disease.Methods1.Collecting and analyzing retrospectively the clinical and pathological data of13 DLBCL/BL patients, who were treated in the First Affiliated Hospital of Zhengzhou University between January 2013 and December 2014. We set up follow up data, and made a count of the survival time and survival status.2.According to first chemotherapy intensity, 11 cases of DLBCL/BL, who underwent chemotherapy, were divided into CHOP or CHOP-like group and intensive therapy group. Survival analysis was performed to explore the relationship between first chemotherapy intensity and treatment response and the prognosis of DLBCL/BL.3.Through univariate analysis, explore the relationship between gender, age,clinical stage, serum lactate dehydrogenase(LDH) level, β2 microglubulin level,international prognostic index(IPI) score, cell of origin, etc and the prognosis of DLBCL/BL.4.Statistical methods: Statistical analysis was performed by SPSS 21.0 software.Survival curve was drawn by Kaplan-Meier method. Univariate analysis was performed by Log-Rank method. For all tests, P≤0.05 was considered statistically significant.Results1.The Clinical characteristic of the 13 patients with DLBCL/BL: The median age was 59 years. The male to female ratio was 1.6:1. Extra-nodal involvement was observed in 12 patients and the sites≥2 in 5 cases. Bone marrow invasion was observed in 4 patients.2.The treatment response and survival analysis: Among the 13 patients with DLBCL/BL, The median OS and PFS were only 10 and 6 months, respectively. The1-year OS and PFS rate were 46.2% and 38.5%, respectively. Among the 11 patients who received chemotherapy, the objective response rate was 81.8% and the complete response was 45.5%. The median overall survival(mOS) of CHOP or CHOP-likegroup and intensive therapy group were 7 months and 13 months, respectively. The median progression-free survival(mPFS) of CHOP or CHOP-like group and intensive therapy group were 4 months and 12 months, respectively. Some borderline differences in survival were noted among the CHOP or CHOP-like, and intensive chemotherapy groups.(OS:P=0.064;PFS:P=0.054)。3.Prognostic factors: Univariate analysis demonstrated that the mOS of the IPI scores ≥3 group and IPI scores <3 group were 7 months and 14 months, respectively.The mPFS of the IPI scores ≥3 group and IPI scores <3 group were 4.5 months and12 months, respectively. The differences were statistically significant(OS:P=0.046;PFS:P=0.030). The mOS of the LDH normal group and LDH elevated group were 17 months and 7 months, respectively. The m PFS of the LDH normal group and LDH elevated group were 12 months and 3.5 months, respectively. The differences were statistically significant(OS:P=0.007;PFS:P=0.007).ConclusionDLBCL/BL is an aggressive B-cell lymphoma with a short survival time. The majority of patients presented extra-nodal involvement. DLBCL/BL did not respond well to CHOP or CHOP-like regimen, and more intensive chemotherapy may improve survival. Elevated LDH levels and high IPI scores were predictors of poor survival. |
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