骨原发性淋巴瘤7例临床分析

骨原发性淋巴瘤（Primary bone lymphoma，PBL）是一种很少见的恶性肿瘤，在结外淋巴瘤中发病率占4％～5％。本文总结我院有病理证实的PBL病例共7例，分析其临床特点、诊断、治疗方法和预后。     

骨原发性恶性淋巴瘤的诊断治疗和预后

恶性淋巴瘤是原发于淋巴结和淋巴结外淋巴组织的恶性肿瘤。根据瘤细胞特点和瘤组织的结构成分，可将恶性淋巴瘤分为以RS细胞为特征的霍奇金病（Hodgkin disease，HD）和非霍奇金淋巴瘤（non-hodgkin lymphoma,NHL）两大类。骨原发性恶性淋巴瘤又称骨原发性非霍奇金淋巴瘤（Primary Non-hodgkin’s Lymphoma of Bone）指起源于骨髓腔的淋巴瘤，同时不伴区域淋巴结或内脏受累，是一种很少见的结外淋巴瘤。     

原发性胃肠道淋巴瘤的诊断治疗进展

原发性胃肠道淋巴瘤(PGIL)是最常见的结外淋巴瘤，其组织发生、病理形态和生物学行为与结内淋巴瘤不同，是一组具有独特临床病理特征的肿瘤，新的分类、分期标准有助于提高临床病理诊断水平。其治疗和预后与结外淋巴瘤及胃肠道癌不同。治疗包括抗幽门螺杆菌治疗、以手术为主的综合治疗和非手术综合治疗，对于Ⅰ、Ⅱ期原发性胃淋巴瘤首选手术还是放、化疗尚存在争议，回顾分析近年来PGIL的诊断、治疗及预后资料，对不同病理分型、分期患者提出不同的治疗原则。     

原发性骨淋巴瘤3例并文献复习

目的：探讨原发性骨淋巴瘤的临床特征、治疗方法及预后.方法：对南京八一医院确诊为原发性骨淋巴瘤的3例患者的临床资料进行回顾性分析.结果：随访8月-11年,采用化疗联合放疗2例,均存活.单纯化疗1例,因病情进展已死亡.结论：原发性骨淋巴瘤为临床少见的结外淋巴瘤,以弥漫大B细胞性非霍奇金淋巴瘤多见,预后较好.通常见为溶骨性破坏,局部症状重而全身症状轻,影像学检查无特异性,造成早期准确诊断非常困难,容易发生误诊.确诊有赖于病理及免疫组化证实.治疗以放化疗综合治疗为宜.     

原发性骨淋巴瘤的影像学及临床病理特征

 原发性骨淋巴瘤（PLB）为非霍奇金淋巴瘤（NHL），是一类罕见的结外淋巴瘤，占所有NHL的1 %、结外淋巴瘤的5 %、原发于骨的恶性肿瘤的7 %。目前公认的PLB诊断标准为：肿瘤局限于单骨，临床和影像学检查未发现有其他系统病灶；病理组织学上确诊骨病灶为淋巴瘤；就诊时只有局部浸润，或至少在原发灶出现6个月后才有远处骨骼和其他部位的转移。由于PLB 表现的多样性和异质性，且发病率较低，导致诊断困难，现分析归纳其临床病理及影像学特征，以提高认识，降低误诊和漏诊率。     

44例原发性结外淋巴瘤临床分析

淋巴瘤是一种起源于淋巴结或其他淋巴组织的恶性肿瘤,组织病理学可见淋巴细胞的肿瘤性增生。本病大多原发于淋巴结内,但10％～44％的原发性结外淋巴瘤,病灶起源于淋巴结外器官,早期易被误诊而延误治疗。为了探讨原发性结外淋巴瘤的临床     

胃淋巴瘤的研究进展

原发性胃淋巴瘤属结外淋巴瘤。临床上容易误诊，现就目前的研究状况做一综述。资料统计，胃肠非霍奇金淋巴瘤大约占全部非霍奇金淋巴瘤发病率的4％～20％，而胃是结外淋巴瘤的好发部位，大约占全部消化道淋巴瘤的55％～65％。原发性胃淋巴瘤绝大部分为B……     

原发性胃肠道淋巴瘤的诊断治疗进展

原发性胃肠道淋巴瘤(PGIL)是最常见的结外淋巴瘤,其组织发生、病理形态和生物学行为与结内淋巴瘤不同,是一组具有独特临床病理特征的肿瘤,新的分类、分期标准有助于提高临床病理诊断水平.其治疗和预后与结外淋巴瘤及胃肠道癌不同.治疗包括抗幽门螺杆菌治疗、以手术为主的综合治疗和非手术综合治疗,对于Ⅰ、Ⅱ期原发性胃淋巴瘤首选手术还是放、化疗尚存在争议,回顾分析近年来PGIL的诊断、治疗及预后资料,对不同病理分型、分期患者提出不同的治疗原则.     

原发性结外淋巴瘤43例报告

目的:探讨原发性结外淋巴瘤的临床病理特点和早期诊断。方法回顾分析我院1996年11月至2007年12月收治的经病理学证实的的43例原发性结外淋巴瘤临床病理资料。结果:原发性结外淋巴瘤无特异性临床特征,内窥镜取材的部位深度不够,B超,CT等影像学检查不能定性,初诊时误诊率达100%。结论:提高临床医生对该病的认识,尽早获取病理学依据,以早期诊断,治疗。     

原发性肺非霍奇金淋巴瘤３例

原发性肺淋巴瘤是指起源于肺内淋巴组织的恶性淋巴瘤,是一种相当少见的结外淋巴瘤,其发病率不足1%,约占结外淋巴瘤的3.6%。我院自2003年以来共收治原发性肺非霍奇金氏淋巴瘤3例,现对其临床表现、影像学特点、支气管镜下表现、诊断及治疗方法进行分析。     

37例原发骨恶性淋巴瘤的临床特点及预后因素分析

探讨原发骨恶性淋巴瘤（primary bone lymphoma,PBL）的临床特点及其与预后的相关性。方法:回顾性分析1995年6月至2009年5月本院收治的37例PTL患者的临床资料,以Kaplan-Meier法绘制生存曲线,用Log-rank检验进行单因素分析,多因素分析采用Cox回归模型以评估独立的预后因素。结果:37例患者的中位发病年龄为61（18～85）岁,首发症状主要表现为骨痛,局部软组织肿胀、肿块形成和病理性骨折。78%患者的病理类型为弥漫大B细胞淋巴瘤。经化疗和/或放疗,18例完全缓解（complete response,CR）,13例部分缓解（partial response,PR）,3例稳定（stable disease,SD）,2例进展（progressive disease,PD）。中位随访时间32（7～171）个月,5年和10年总生存率分别为59.5%和43.2%。患者接受4周期以上化疗,B细胞淋巴瘤加用利妥昔单抗者疗效较好。多因素分析显示:Ann Arbor分期、B症状、年龄和结外受侵数是PBL的独立预后因素。结论:PBL应采取综合治疗,同时给予蒽环类药物为主的全身化疗,B细胞淋巴瘤首选利妥昔单抗联合化疗,给予帕米膦酸盐治疗骨病变。Ann Arbor分期、B症状、年龄和结外受侵数为PBL预后的独立影响因素。      

原发性乳腺恶性淋巴瘤11例临床分析

为了探讨原发乳腺恶性淋巴瘤(PBL)的临床特点、病理特征及预后影响因素,对11例PBL患者的临床特点及病理类型应用Kaplan-Meier法进行生存分析.本组病例中部位、白蛋白水平、分期、放疗和手术5个因素影响患者预后.双侧痛变患者的生存率较单侧病变者低,P=0.005.监测患者首治前白蛋白水平,伴有白蛋白下降的患者生存率低,两组间差异有统计学意义,P=0.018.11例随着分期的增加,患者的预后也越差,P=0.040.全组中有5例患者接受放射治疗,未接受放疗的患者生存率比放疗者低,P=0.020.首诊进行手术的患者为9例,其与未手术组间差异有统计学意义,P=0.002.初步研究结果提示,PBL是一组罕见的结外淋巴瘤,临床表现不典型,白蛋白水平低和双侧原发患者的预后差,治疗以手术联合化放疗为佳.     

Incidence and pathology of primary brain lymphoma in Hong Kong Chinese patients

Primary brain lymphoma (PBL) is an uncommon extranodal lymphoma. Its incidence is rapidly increasing in both immunocompromised and immunocompetent patients in Western countries. Eighteen cases of PBL were identified during a 16-year period among HIV negative patients in Queen Mary Hospital, Hong Kong. One case of post-transplantation lymphoproliferative disease (PTLD) was positive for Epstein Barr virus (EBV) encoded RNA (EBER) by in situ hybridization. All the remaining 17 immunocompetent cases were classified as diffuse large B-cell lymphoma, except for one case of Burkitt's lymphoma. EBER expression was negative in all 13 cases tested. Immunostaining for bcl-2 and bcl-6 was positive in 8/11 and 6/11 cases tested, with heterogeneous combination of expression and intensity. The incidence rate of PBL in immunocompetent patients was stable at 1.03 per million per year. The incidence of PBL in post transplantation (0.16%) and HIV related setting (0.29%) is also low in Chinese. PBL in Chinese patients is almost uniformly represented by EBV negative, diffuse large B-cell lymphoma, confined to the brain. However, the molecular pathogenesis may be heterogeneous.     

原发乳腺弥漫大B细胞淋巴瘤复发1例诊治探讨

原发性乳腺淋巴瘤是一种少见的结外淋巴瘤,预后相对较差,目前尚无统一治疗方案,现介绍1例经本院诊治的反复复发的原发乳腺弥漫大B细胞淋巴瘤病例,该患者经过手术、化疗、靶向药物治疗、放疗等多专业联合诊治获得了良好的治疗效果。通过对本例患者诊治进行多学科讨论,进一步探讨原发乳腺淋巴瘤的规范化诊疗流程,推动多学科间的合作。      

Primary non-Hodgkin malignant lymphoma of the breast. An immunohistochemical study of seven patients and literature review of 152 patients with breast lymphoma in Japan.

BACKGROUND. The breast is rarely a primary site for extranodal malignant lymphoma. Most reported primary non-Hodgkin malignant lymphomas of the breast (PBL) are of B-cell phenotype. METHODS. Histologic and immunohistochemical analyses of seven patients with PBL and a statistical analysis of 152 patients with PBL reported in the Japanese literature were performed. RESULTS. Malignant lymphoma could not be predicted preoperatively with clinical and radiologic findings; breast carcinoma, fibroadenoma, and phyllodes tumor were the preoperative diagnoses. All patients were women; they ranged in age from 31 to 80 years (mean, 57.6 years). The right breast was involved initially in five patients. In four, only the breast was involved (Stage I), whereas in three, the ipsilateral axillary lymph nodes (Stage II) were involved at diagnosis. According to the Working Formulation, all patients belonged to the intermediate grade and were classified as having diffuse large cell (five patients) or mixed (two patients) lymphoma. Immunophenotypic analysis revealed that all patients had B-cell lymphoma. No patients had lymphoepithelial lesions, which is the characteristic feature in categorizing a lymphoma as a mucosa-associated lymphoid tissue (MALT) lymphoma. A statistical analysis of the patient reported in the Japanese literature has divided PBL into two types: a bilateral type that affects younger women and a unilateral type that has a broad age distribution, but preponderantly occurs in older women. The age and stage at diagnosis were significant prognostic factors in predicting the survival time, but the location and size of the tumor at initial presentation, histopathologic type, terminal leukemic manifestation, and treatment modality were not. CONCLUSIONS. This study indicates that most PBL are diffuse large cell lymphoma of B-cell phenotype and that the age and stage at diagnosis are significant prognostic factors.     

青海地区淋巴瘤253例临床特点与预后因素分析

目的 分析淋巴瘤的临床特点及预后相关因素.方法 对青海地区253例淋巴瘤患者临床资料进行回顾性研究并随访.采用Kaplan-Meier法分析患者不同临床特点及实验室检查的生存期差异.采用Cox比例风险回归模型分析影响预后的因素.结果 253例淋巴瘤患者中,男、女比例为1.56∶1,中位年龄为48岁,发病年龄高峰为40岁和60岁左右.结内起病（56.13％）较多,结外起病常见部位为鼻咽部和胃肠道.病理类型为霍奇金淋巴瘤40例（15.81％）,非霍奇金淋巴瘤213例（84.19％）.前者以结节硬化型（72.50％）最多见;后者中B细胞淋巴瘤148例（69.48％）,最常见的病理类型为弥漫大B细胞淋巴瘤（39.91％）、滤泡细胞淋巴瘤（12.21％）及周围T细胞非特殊型淋巴瘤（9.39％）.单因素预后分析显示,临床分期、有无B症状、血红蛋白（Hb）、乳酸脱氢酶（LDH）、国际预后指数（IPI）及治疗方案与非霍奇金淋巴瘤预后密切相关（均P＜0.05）.Cox回归模型多因素分析显示,临床分期、IPI及治疗方案与非霍奇金淋巴瘤的预后关系密切,差异有统计学意义（均P＜0.05）.结论 青海地区淋巴瘤发病年龄有两个高峰,结内发病高于结外,B细胞淋巴瘤多见,IPI分组适用于该地区非霍奇金淋巴瘤的预后评估,并可作为其独立的预后因素.     

Importance of combined-modality therapy for primary bone lymphoma

Primary bone lymphoma (PBL) is a rare entity and comprises about 5% of all extranodal non-Hodgkin's lymphomas (NHL) and 7% of all primary bone tumors. To date there is no consensus about the optimal treatment for PBL. We retrospectively reviewed all cases of PBL treated at Hospital S?o Paulo, Brazil, over a 10-year-period (January 1992-January 2002). Medical records of 7 patients with PBL were reviewed and information on age at diagnosis, sex, NHL clinical staging (CS), treatment and response to treatment were retrieved. Five patients (72%) received combined-modality therapy (CMT) and all of them are in complete remission (CR) with a median follow up of 19 months (ranging from 12 to 144 months). We conclude that PBL is a potentially curable malignancy and treatment should be undertaken in a multiprofessional approach, in order to provide the best support which probably has to include chemotherapy, radiotherapy and, for patients with IPI higher than 2, consolidation with stem-cell transplantation.     

Analysis of differential therapeutic strategies for primary breast lymphoma: two case reports

Primary breast lymphoma (PBL) is a rare form of extranodal non-Hodgkin’s lymphoma (NHL), whose own specific biological characteristics still need to be fully defined. No significant prognostic factor has been found and the optimal therapeutic strategy is uncertain. However, an intensified systemic therapy has been advocated to prevent relapse, even in patients who show a complete response to local treatment. We report two cases of primary diffuse large B-cell breast lymphoma, review the literature about this topic, and discuss treatment options. We conclude that differential therapeutic strategies based on the risk of relapse associated with the International Prognostic Index (IPI) are a reasonable way to approach PBL, and can avoid undue toxicity deriving from treatment.     

Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma: a single institutional experience and literature review

Plasmablastic lymphoma (PBL) is a rare aggressive B-cell lymphoproliferative disorder. HIV-negative PBL has not been extensively reported. Nine HIV-negative PBL patients evaluated at Moffitt Cancer Center were studied. Eight patients had extranodal diseases. All patients were treated with CHOP or hyper-CVAD. Responses were observed in 8 cases (7 complete, 1 partial responses). Four patients underwent consolidation with autologous hematopoietic stem cell transplant (HSCT) in first complete remission (CR1). At median follow-up of 23.9 months, 7 patients were alive and 5 were disease-free. Aggressive induction chemotherapy and consolidation with autologous HSCT in CR1 might be considered for patients with HIV-negative PBL.     

Esophageal involvement by extranodal natural killer T cell lymphoma, nasal type, mimicking Ebstein Barr viral esophagitis in a tonsillar lymphoma patient undergoing chemoradiation therapy

Esophageal involvement by extranodal natural killer (NK)/T cell lymphoma, nasal type, is rare. As a result, esophageal symptoms in these patients might at first be thought to originate from a benign condition, such as viral esophagitis. It is important to note, however, that benign conditions may mask esophageal involvement by lymphoma. Until now, there has been no report documenting esophageal involvement by lymphoma mimicking viral esophagitis in an extranodal NK/T cell lymphoma patient undergoing active treatment. Here, we report a case of esophageal involvement by extranodal NK/T cell lymphoma, nasal type, initially misdiagnosed as Ebstein Barr virus esophagitis. Lymphoma invasion of the esophagus should be considered if esophageal symptoms do not respond to usual medical esophagitis therapy in an extranodal NK/T cell lymphoma, nasal type, patient undergoing chemoradiation.