原发性骨淋巴瘤（primary bone lymphoma,PBL）是一种罕见的结外淋巴瘤,仅占结外非霍奇金淋巴瘤的4%~5%,约占骨原发肿瘤的7%[1]。由于该病发病率低,临床认识不足,导致该病的早期诊断、规范化治疗及预后评估较为困难。现将我院收治的1例...原发性骨淋巴瘤（primary bone lymphoma,PBL）是一种罕见的结外淋巴瘤,仅占结外非霍奇金淋巴瘤的4%~5%,约占骨原发肿瘤的7%[1]。由于该病发病率低,临床认识不足,导致该病的早期诊断、规范化治疗及预后评估较为困难。现将我院收治的1例PBL患者的诊治过程报告如下。展开更多
目的:本文旨在探讨回肠巨大原发性弥漫性大B细胞淋巴瘤的临床表现、影像学特点及诊治要点。方法:对延安市人民医院胃肠外科收治的回肠巨大原发性弥漫性大B细胞淋巴瘤合并肠梗阻1例患者进行病例回顾并复习相关文献。结果:回肠巨大原发性...目的:本文旨在探讨回肠巨大原发性弥漫性大B细胞淋巴瘤的临床表现、影像学特点及诊治要点。方法:对延安市人民医院胃肠外科收治的回肠巨大原发性弥漫性大B细胞淋巴瘤合并肠梗阻1例患者进行病例回顾并复习相关文献。结果:回肠巨大原发性弥漫性大B细胞淋巴瘤,发病率低,临床表现不典型;合并肠梗阻者,往往容易误诊及漏诊,最终确诊要需手术探查及病理。结论:回肠原发性弥漫性大B细胞淋巴瘤合并肠梗阻缺乏特异性临床表现,手术为首选治疗方法,需结合手术病理及免疫组化进行确诊。Objective: To explore the clinical manifestations, imaging characteristics, and key points of diagnosis and treatment of giant primary diffuse large B-cell lymphoma in the ileum. Method: A case review and literature review were conducted on a patient with giant primary diffuse large B-cell lymphoma of the ileum and intestinal obstruction admitted to the Department of Gastroenterology at Yan’an People’s Hospital. Results: The large primary diffuse large B-cell lymphoma of the ileum had a low incidence rate and atypical clinical manifestations;Patients with combined intestinal obstruction are often prone to misdiagnosis and missed diagnosis, and the final diagnosis requires surgical exploration and pathology. Conclusion: Primary diffuse large B-cell lymphoma of the ileum with intestinal obstruction lacks specific clinical manifestations, and surgery is the preferred treatment method. Diagnosis should be based on surgical pathology and immunohistochemistry.展开更多
目的探讨原发性骨弥漫性大B细胞淋巴瘤(primary bone diffuse large B-cell lymphoma,PB-DLBCL)的临床病理特征、突变图谱及预后影响因素。方法纳入2006年5月至2021年9月上海交通大学医学院附属瑞金医院收治的初治PB-DLBCL患者37例。采...目的探讨原发性骨弥漫性大B细胞淋巴瘤(primary bone diffuse large B-cell lymphoma,PB-DLBCL)的临床病理特征、突变图谱及预后影响因素。方法纳入2006年5月至2021年9月上海交通大学医学院附属瑞金医院收治的初治PB-DLBCL患者37例。采用淋巴瘤相关55个基因靶向测序描绘PB-DLBCL患者的突变图谱,采用单因素Cox回归模型评估临床因素和基因突变与无进展生存期(PFS)和总生存期(OS)之间的关系。结果37例初治PB-DLBCL患者中,男性21例(56.8%),年龄>60岁21例(56.8%),Ann Arbor分期为Ⅳ期22例(59.5%),非生发中心来源(non-GCB)亚型22例(59.5%),国际预后指数评分为3~5的患者18例(48.6%)。靶向测序结果显示,MYD88、PIM1、CCND3、CD79B、CIITA、HIST1H1E、KMT2C、PRDM1、TNFAIP3、ZFP36L1为常见突变,突变频率高于20%。单因素分析结果显示,BTG2基因突变(P=0.015)、MYD88基因突变(P=0.049)及MYC/BCL2双表达淋巴瘤(DEL,P=0.009)与PB-DLBCL患者低PFS率显著相关。结论PB-DLBCL患者中non-GCB亚型较为常见,DEL,BTG2和MYD88基因突变是PB-DLBCL患者PFS的不良影响因素。展开更多
原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是指起源于颅内、眼、脊髓和软脑膜的非霍奇金淋巴瘤^([1]),且肿瘤在诊断后6个月内未累及中枢神经系统以外的器官或组织。自1991年由Lachance等首次提出以来,...原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是指起源于颅内、眼、脊髓和软脑膜的非霍奇金淋巴瘤^([1]),且肿瘤在诊断后6个月内未累及中枢神经系统以外的器官或组织。自1991年由Lachance等首次提出以来,国内外对PCNSL进行了广泛的研究。PCNSL约占非霍奇金淋巴瘤(non-Hodgkin lymphoma,NHL)的3%,占所有脑肿瘤的2%~3%^([1-2]),年发病率为(0.4~0.5)人/10万,70岁以上老年人的年发病率增至4人/10万,先天或后天获得性免疫缺陷患者PCNSL的发病率远高于免疫正常者,而国内报道的病例多为后者。展开更多
文摘非霍奇金淋巴瘤(non-Hodgkin lymphoma,NHL)是临床常见的肿瘤类型,临床表现多样,确诊依赖组织活检,根据病灶起源可以分为结内和结外淋巴瘤。原发性胃肠淋巴瘤是结外淋巴瘤的常见部位,可以累及胃、小肠、结肠等,其中约50%~60%发生在胃部,病理类型以黏膜相关淋巴组织淋巴瘤和弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)为主[1~3]。
文摘目的:本文旨在探讨回肠巨大原发性弥漫性大B细胞淋巴瘤的临床表现、影像学特点及诊治要点。方法:对延安市人民医院胃肠外科收治的回肠巨大原发性弥漫性大B细胞淋巴瘤合并肠梗阻1例患者进行病例回顾并复习相关文献。结果:回肠巨大原发性弥漫性大B细胞淋巴瘤,发病率低,临床表现不典型;合并肠梗阻者,往往容易误诊及漏诊,最终确诊要需手术探查及病理。结论:回肠原发性弥漫性大B细胞淋巴瘤合并肠梗阻缺乏特异性临床表现,手术为首选治疗方法,需结合手术病理及免疫组化进行确诊。Objective: To explore the clinical manifestations, imaging characteristics, and key points of diagnosis and treatment of giant primary diffuse large B-cell lymphoma in the ileum. Method: A case review and literature review were conducted on a patient with giant primary diffuse large B-cell lymphoma of the ileum and intestinal obstruction admitted to the Department of Gastroenterology at Yan’an People’s Hospital. Results: The large primary diffuse large B-cell lymphoma of the ileum had a low incidence rate and atypical clinical manifestations;Patients with combined intestinal obstruction are often prone to misdiagnosis and missed diagnosis, and the final diagnosis requires surgical exploration and pathology. Conclusion: Primary diffuse large B-cell lymphoma of the ileum with intestinal obstruction lacks specific clinical manifestations, and surgery is the preferred treatment method. Diagnosis should be based on surgical pathology and immunohistochemistry.
文摘目的探讨原发性骨弥漫性大B细胞淋巴瘤(primary bone diffuse large B-cell lymphoma,PB-DLBCL)的临床病理特征、突变图谱及预后影响因素。方法纳入2006年5月至2021年9月上海交通大学医学院附属瑞金医院收治的初治PB-DLBCL患者37例。采用淋巴瘤相关55个基因靶向测序描绘PB-DLBCL患者的突变图谱,采用单因素Cox回归模型评估临床因素和基因突变与无进展生存期(PFS)和总生存期(OS)之间的关系。结果37例初治PB-DLBCL患者中,男性21例(56.8%),年龄>60岁21例(56.8%),Ann Arbor分期为Ⅳ期22例(59.5%),非生发中心来源(non-GCB)亚型22例(59.5%),国际预后指数评分为3~5的患者18例(48.6%)。靶向测序结果显示,MYD88、PIM1、CCND3、CD79B、CIITA、HIST1H1E、KMT2C、PRDM1、TNFAIP3、ZFP36L1为常见突变,突变频率高于20%。单因素分析结果显示,BTG2基因突变(P=0.015)、MYD88基因突变(P=0.049)及MYC/BCL2双表达淋巴瘤(DEL,P=0.009)与PB-DLBCL患者低PFS率显著相关。结论PB-DLBCL患者中non-GCB亚型较为常见,DEL,BTG2和MYD88基因突变是PB-DLBCL患者PFS的不良影响因素。
文摘原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是指起源于颅内、眼、脊髓和软脑膜的非霍奇金淋巴瘤^([1]),且肿瘤在诊断后6个月内未累及中枢神经系统以外的器官或组织。自1991年由Lachance等首次提出以来,国内外对PCNSL进行了广泛的研究。PCNSL约占非霍奇金淋巴瘤(non-Hodgkin lymphoma,NHL)的3%,占所有脑肿瘤的2%~3%^([1-2]),年发病率为(0.4~0.5)人/10万,70岁以上老年人的年发病率增至4人/10万,先天或后天获得性免疫缺陷患者PCNSL的发病率远高于免疫正常者,而国内报道的病例多为后者。
