原发性骨弥漫大B细胞淋巴瘤15例临床病理特征及预后  

Clinicopathological characteristics and prognosis of primary bone diffuse large B cell lymphoma:report of fifteen cases

作　　者：梁小芹[1] 苏勤军 冯友繁[3] 王慧春[4] 王卓[1] 王金穗[1] LIANG Xiao-qin;SU Qin-jun;FENG You-fan;WANG Hui-chun;WANG Zhuo;WANG Jin-sui(Department of Pathology,Gansu Province People’s Hospital,Lanzhou 730000,China;Department of Pathology,the 940 Hospital of PLA Joint Logistics Support Force,Lanzhou 730050,China;Department of Hematology,Gansu Province People’s Hospital,Lanzhou 730000,China;PET/CT Center,Gansu Province People’s Hospital,Lanzhou 730000,China)

机构地区：[1]甘肃省人民医院病理科,兰州730000 [2]中国人民解放军联勤保障部队第九四〇医院病理科,兰州730050 [3]甘肃省人民医院血液科,兰州730000 [4]甘肃省人民医院PET/CT中心,兰州730000

出　　处：《临床与实验病理学杂志》2021年第12期1455-1459,1464,共6页Chinese Journal of Clinical and Experimental Pathology

基　　金：甘肃省人民医院院内科研基金(18GSSY4-38)。

摘　　要：目的探讨原发性骨弥漫大B细胞淋巴瘤(primary bone diffuse large B-cell lymphoma,PB-DLBCL)的临床病理特征、治疗及预后。方法收集15例PB-DLBCL的临床资料,对其行免疫组化EnVision两步法和荧光原位杂交检测,总结其临床病理特征、治疗及预后。结果患者临床首发症状为局部疼痛,病变累及颌骨6例(40%)、扁骨5例(33.3%)、长骨4例(26.7%)。镜下见大淋巴细胞样瘤细胞弥漫分布于骨小梁,瘤细胞浸润性生长,核仁较明显,分裂象易见。免疫表型:6例为生发中心型,9例为非生发中心型;15例EBER均阴性;15例PB-DLBCL中2例c-myc基因断裂重排,1例BCL-2基因断裂重排,3例BCL-6基因断裂重排。Ann Arbor临床分期:Ⅰ期8例,Ⅱ期2例,Ⅳ期5例。15例患者中,1例确诊后未治疗,3例行单纯手术治疗,11例行CHOP及相关方案治疗;8例完全缓解,4例部分缓解,1例病情稳定,1例病情进展。随访1~38个月,中位随访时间19个月,10例患者存活,4例死亡,1例失访。结论PB-DLBCL的临床表现及影像学检查缺乏特异性,诊断需结合病理形态学改变、免疫表型及分子检测以提高诊断准确性,并进行及时规范化治疗以提高患者生存率。Purpose To investigate the clinicopathologicl characteristics,diagnosis,efficacy and prognosis of primary bone diffuse large B cell lymphoma(PB-DLBCL).Methods The clinical data of 15 patients with PB-DLBCL were retrospectively analyzed,and clinical/patholgical characteristics,treatment,as well as prognosis were summarized.Results The primary clinical symptoms of all patients were pain at the lesion site.It occurred mainly in the jaw(6,40%),flat bones(5,33.3%)and long bones(4,26.7%).The tumor cells with high mitotic activity were large-size pleomorphic lymphoid cells which diffusely infiltrated in bone trabeculae.Immunophenotyping showed 6 cases were GCB,and 9 cases were of non-GCB type.All of patients were negative for EBER.In 2 cases,c-myc gene carried broken mutation.1 cases had BCL-2 gene rearrangement,and 3 case had BCL-6 gene rearrangement.According to Ann Arbor staging,8 cases were stage Ⅰ,2 cases Ⅱ,and 5 cases stage Ⅳ.Among the 15 diagnosed PB-DLBCL,1 patient underwent no treatment,3 patients surgery alone,11 patients were treated with CHOP related regimens.8 patients got complete remission,4 patients partial remission,1 patient stable disease and 1 patient progressive disease respectively.The follow-up period was 1-38 months,and the median follow-up time was 19 months,10 patients survived,4 patients died,and 1 case was lost to follow-up.Conclusion PB-DLBCL is a rare malignant tumor with relatively good prognosis.Clinical and imaging features lack characteristics.Its diagnosis is mainly confirmed by combined pathomorphologic changes with immunophenotype and molecular detection.In addition,timely and standardized therapy may improve survival rate.

关 键 词：骨肿瘤 弥漫大B细胞淋巴瘤 免疫组织化学 治疗 预后 

分 类 号：R733.4[医药卫生—肿瘤]