原发性乳腺非霍奇金淋巴瘤20例临床分析

目的:探讨原发性乳腺非霍奇金淋巴瘤(NHL)的临床特点、诊治及其预后。方法:对20例原发性乳腺非霍奇金淋巴瘤患者的临床资料进行回顾性分析。结果:20例原发性乳腺非霍奇金淋巴瘤患者中,女19例,男1例,中位年龄38岁(25~76岁),其中Ⅰ期9例(45.0%),Ⅱ期7例(35.0%),Ⅲ期3例(15.0%),Ⅳ期1例(5.0%)。1例(5.0%)为未化分型NHL,19例(95.0%)为B细胞性NHL。总的3、5年生存率分别为85.0%、65.0%,Ⅰ期 Ⅱ期3、5年生存率分别为93.75%、81.25%。结论:原发性乳腺非霍奇金淋巴瘤的预后与分期和治疗方式有关,手术 化疗综合治疗可获得较好疗效。     

粘膜相关淋巴组织(MALT)型淋巴瘤的临床特点和处理

粘膜相关淋巴组织(MALT)型淋巴瘤是非霍奇金恶性淋巴瘤中一个独立的亚型，有其独特的临床病理特点。它是结外低度恶性B细胞淋巴瘤中最常见的一型。此型淋巴瘤病人常有慢性感染病史或自身免疫性疾病。MALT淋巴瘤是一种惰性病变，可较长时间局限于局部。局部治疗有效。胃MALT淋巴瘤最常见。本文以胃MALT淋巴瘤为重点，对MALT淋巴瘤的临床特点和处理作一个综述。     

Primary mucosa-associated lymphoid tissue lymphoma of the breast

Mucosa-associated lymphoid tissue (MALT) lymphoma is an extranodal indolent lymphoma with histopatholigic features similar to those of marginal zone B-cell lymphomas. Primary breast MALT lymphomas were first described by Lamovec and Jancar as a low-grade B-cell lymphoma in 1987. Herein, a case is presented of a patient with primary MALT lymphoma of the breast. Issues in diagnosis and breast-conservation treatment, as it pertains to primary MALT lymphoma of the breast, will be discussed.     

外科手术在原发肺黏膜相关淋巴瘤中的诊治作用

  目的  探讨原发肺黏膜相关淋巴组织(MALT)淋巴瘤的临床特点、影像学特征、治疗方法及其预后。  方法  回顾性分析解放军总医院2000年4月至2012年7月收治的13例肺MALT淋巴瘤患者的临床、影像学和随访资料。  结果  13例肺MALT淋巴瘤患者中,男8例,女5例,男女比例1.6∶1;发病年龄21~67岁,中位年龄59岁。主要临床表现为胸部不适6例,咳嗽2例,发热2例,胸痛1例,还有2例无明显不适症状;有1例合并有干燥综合征。胸部CT表现为3例有肺实变影伴有支气管充气征,3例有斑片影,4例中出现团块影,还有3例可见磨玻璃结节影,其中10例患者表现为双肺均有病变,另有4例伴有纵隔淋巴结肿大,1例伴有胸腔积液。6例行手术治疗,其中3例术后辅以化疗,4例患者单纯行化疗,方案主要为CHOP方案或R-CHOP方案,3例患者仅行对症支持治疗。13例中1例失访,随访时间1~11年。1例于确诊4年后出现疾病进展,并于11年后死亡,1例因化疗不良反应于确诊后2年内死亡,余10例至今仍生存,且无疾病进展。  结论  肺MALT淋巴瘤的临床表现不典型,影像学缺乏特异性改变,确诊依靠病理学检查。对于那些病变局限,尤其是常规方法无法取得病理的患者,手术切除病灶既能明确诊断,又能使患者获得良好的治疗效果,在该病的诊治过程中起到了重要的作用。该病预后较好。      

黏膜相关淋巴组织淋巴瘤治疗研究进展

黏膜相关淋巴组织淋巴瘤是起源于淋巴结外的低度恶性淋巴瘤,是最常见的边缘区B细胞淋巴瘤(MZL),约占惰性非霍奇金淋巴瘤(NHL)的30％～50％.其发病部位广泛,治疗方法多样.现就近年来黏膜相关淋巴组织淋巴瘤的治疗研究进展进行综述.     

胃黏膜相关淋巴组织淋巴瘤的临床治疗观察

目的：提高对胃黏膜相关淋巴组织（mucosa-associated lymphoid tissue,MALT）淋巴瘤的认识和诊疗水平。方法：回顾性总结我院10年间115例胃黏膜相关淋巴组织淋巴瘤资料,手术标本重新病理诊断,明确分期,并进行追踪随访。结果：98例经手术治疗,17例内科非手术治疗,胃MALT淋巴瘤的病变累计范围较广,胃窦、胃体、小弯侧多见形态多样,腹腔淋巴结易受累。病理属低度恶性,预后良好。结论：胃MALT淋巴瘤是一类新型淋巴瘤,与幽门螺旋菌感染有关,及时有效治疗,预后较好,生存期可延长。     

胃黏膜相关淋巴组织淋巴瘤临床分析

 目的 探讨胃黏膜相关（MALT）淋巴瘤的临床特点和合理治疗方法。方法 回顾性分析总结10例胃MALT淋巴瘤临床资料,包括临床表现、胃镜、胃活组织病理检查、免疫组化检查及幽门螺杆菌（HP）检测和治疗方法。结果 10例均有上腹部不适或疼痛,其中6例伴有黑便。胃镜检查均见病灶处分布大小不等结节状隆起,病灶质地僵硬,接触易出血,7例见病灶处分布大小不等溃疡。6例病灶位于胃窦,2例位于胃角,2例位于胃体;胃活组织病理检查均显示胃黏膜组织中间质内有大量小淋巴细胞弥漫性浸润,浸润淋巴滤泡的边缘带,取代和破坏部分胃黏膜腺体和上皮成分。免疫组化：LCA（+）,CD+20,CD+79a,bcl-2（+）,CD-3,CD-5,CD-10,KP-1（-）,Ki-67（-）。HP检测7例阴性,3例阳性。治疗：5例手术+化疗,2例单纯化疗,2例单纯手术,1例单纯抗HP治疗。除2例手术治疗患者失访外,其余8例至今均处于完全缓解期（CR）,CR时间7 ~ 36个月,中位CR时间17.8个月。结论 胃MALT淋巴瘤临床表现不典型,其发生和HP感染密切相关,诊断主要依据病理和免疫组化检查,其显示免疫表型以结外边缘带B细胞呈单克隆浸润为特点,抗HP治疗是有效治疗方法。     

原发睾丸非霍奇金淋巴瘤26例临床分析

目的：探讨原发睾丸非霍奇金淋巴瘤(non-Hodgidn’s lymphoma,NHL)的临床特点、治疗方法。方法：收集我院1980年10月至2002年2月收治的睾丸NHL26例,Ann Arbor分期ⅠE期17例,ⅡE期6例,ⅣE期3例。全部手术治疗。首程术后化疗24例,以CHOP方案为主,3～6周期,化疗加放疗9例,其中对盆腔／腹主动脉旁／阴囊等区域进行预防性放疗7例,针对病灶区放疗2例。阴囊区用9～12M eV电子线,其余用6～8MV-X线照射。照射剂量范围在36～50Gy,预防照射的平均剂量为40Gy。单纯手术和手术加放疗各1例。结果：全组一、三、五年总体生存率和无进展生存率分别为96．0％、78．1％、52．0％和70．2％、55．3％、49．2％。总失败率为53．8％,其中对侧睾丸、中枢神经系统受侵率分别为15．4％、11．5％,腹膜后淋巴转移率19．2％。结论：睾丸NHL一般为中-高度恶性,结外器官和淋巴结受侵率高,睾丸和腹膜后区分别占结外器官和淋巴结受侵的首位。所有期别的睾丸NHL都应化疗。ⅠE、ⅡE期病例应常规行腹主动脉旁、同侧髂血管旁和阴囊区预防性照射。ⅢE、ⅣE期以化疗为主,残留病灶辅以局部放疗。     

Synchronous mucosa-associated lymphoid tissue (MALT) lymphomas involving bilateral orbits and breasts: a rare clinical entity

Primary orbital and primary breast lymphomas comprise very small subgroups of extranodal lymphomas. Clinical presentation at both these sites together is extremely rare. We describe a case of bilateral orbital and bilateral breast mucosa-associated lymphoid tissue (MALT) lymphomas with bilateral pre-auricular lymph nodal metastasis. The case history, staging and management for this unusual entity are discussed.     

Primary Breast mucosa-associated lymphoid tissue (malt) lymphoma with high-grade transformation evidenced by prominent lymphoepithelial lesions

Primary breast lymphoma, particularly primary mucosa-associated lymphoid tissue (MALT) lymphoma, is a rare disease. We report here a case of a MALT lymphoma of the breast with high-grade transformation. An 84-year-old woman presented with a Pagetoid mass in her right breast. After a clinical diagnosis of breast cancer, T2N1M0, stage IIB, she underwent mastectomy with axillary lymph node dissection. She had no history of autoimmune disease such as Hashimoto thyroiditis or Sjogren disease. Pathologically the tumor tissue was composed of small to large lymphoid cells. The large cells contained a considerable number of centroblasts, forming a sheet-like proliferation centrally. Among the small cells many small cleaved cells were present, which were more predominant in the peripheral areas. Immunohistochemistry revealed that these cells were positive for CD20 and CD79a. The tumor cells infiltrated the ductular epithelial cells, distorting the duct structures, to form lymphoepithelial lesions. Immunohistochemistry for cytokeratin and CD20 was helpful for identification. No germinal centers were formed. MALT lymphoma with high-grade transformation was diagnosed. Rituximab (anti-CD20 antibody) was prescribed as systemic treatment without chemotherapy or irradiation. After 18 months, no tumor recurrence was observed. We emphasize the importance of lymphoepithelial lesions for the diagnosis of MALT lymphoma of the breast.     

早期肺原发黏膜相关淋巴组织淋巴瘤的预后分析及文献回顾

目的 分析早期肺原发黏膜相关淋巴组织(MALT)淋巴瘤的临床特征、治疗方式和预后因素。方法 回顾分析2001—2013年32例早期肺MALT淋巴瘤的临床资料、疗效及生存情况。中位年龄56岁,Ⅰ_E期23例、Ⅱ_E期9例;边缘区B细胞淋巴瘤预后指数(MZLPI)评分0分23例、1分9例。9例接受放疗,8例接受单纯手术,3例手术＋化疗,12例单纯化疗。Kaplan-Meier法计算OS、PFS,Logrank法检验和单因素分析。结果 5年样本数22例。5年OS率为85%,5年PFS率为73%。放疗总缓解9/9,其中CR为6/9,PR为3/9。单因素分析显示未放疗为PFS的影响因素,放疗与未放疗患者5年PFS分别为100%和63%(P=0.029),5年OS分别为100%和79%(P=0.129)。年龄>60岁、ECOG评分2分及MZLPI评分1分为PFS及OS的影响因素(P=0.041、0.018、0.044及P=0.001、0.001、0.003)。结论 早期肺MALT淋巴瘤的预后因素包括年龄、ECOG及MZLPI评分。予以受累野低剂量(24~30 Gy)放疗能提高LC率进而延长生存。     

Immunomodulatory treatment for mucosa-associated lymphoid tissue lymphoma (MALT lymphoma)

The pathogenesis of mucosa-associated lymphoid tissue (MALT) lymphoma has been characterized as a dynamic process driven by lymphoma cell dependency on T-cell signaling, chronic antigenic stimulation of marginal zone B-cells and activation of the nuclear factor-kappa B signaling pathway. This concept is underlined by the strong causal connection of chronic Helicobacter pylori associated gastritis and MALT lymphoma development based on perpetual auto-antigenic stimulation of Helicobacter pylori-specific T-cells, but also its association with further potential infectious triggers and autoimmune disorders for extragastric lymphoma sites. Thus, given the dependency of MALT lymphoma cells on the tumor microenvironment, this specific entity appears highly suitable for immunomodulatory treatment strategies. Several approaches have been assessed in the last years including promising data on immunomodulatory agents “IMiDs” thalidomide and lenalidomide, macrolide antibiotics and antibodies. The aim of the present review is to discuss rationales for immunomodulatory therapies in MALT lymphoma and to present the statu quo on immunomodulatory and therefore chemotherapy-free treatment strategies for these patients.     

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue arising in the lateral ventricle

Extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) lymphomas are a well-described type of low-grade B-cell non-Hodgkin lymphoma. They typically arise adjacent to mucosal surfaces in the gastrointestinal tract, lung and conjunctiva, and, less frequently, in the skin, salivary gland and thyroid gland. Unusual locations, such as the genitourinary tract, thymus and meninges, have also been reported. We recently encountered a case of an intracranial MALT lymphoma in a 53-year-old man who presented with persistent headaches and a seizure. The lesion developed as a mass within the lateral ventricle, appeared to be arising from the choroid plexus, and was not associated with meninges. Histologically, there was a vaguely nodular, dense lymphoid infiltrate with occasional benign follicles colonized by marginal zone lymphoma, suggesting derivation from a focus of prior inflammation. Translocations involving the MALT1 gene were not identified but karyotypic evaluation highlighted a complex cytogenetic profile with many chromosomal abnormalities. This rare case provides insight into the pathophysiology of MALT lymphomas.     

合并单克隆免疫球蛋白血症的黏膜相关淋巴组织淋巴瘤临床特征分析

目的 探讨单克隆免疫球蛋白(M蛋白)血症在黏膜相关淋巴组织淋巴瘤(MALToma)中的发生率及患者临床特征.方法 回顾性分析2010年10月至2015年10月接受M蛋白检测的16例住院MALToma患者的临床资料.结果 16例MALToma患者中,6例初诊时合并M蛋白血症.其中4例为男性,中位年龄56岁(25～82岁),肿瘤原发部位多为胃肠道(4例),临床分期多为Ⅰ～Ⅱ期(4例),国际预后指数(IPI)评分多为0～2分(5例).6例合并M蛋白血症的患者中有4例分泌单克隆性IgM(3例为κ轻链,1例为λ轻链),2例分泌单克隆性IgG(均为κ轻链).6例合并M蛋白血症的患者中有4例伴有浆细胞分化(PCD),10例未合并M蛋白血症的患者有1例伴有PCD(P=0.036).此外,3例合并M蛋白血症的患者在获得疾病缓解的同时M蛋白水平均较初诊时明显下降,其中2例降至正常水平.结论 M蛋白血症可能在MALToma患者中较常见,可能与伴有PCD有关,且M蛋白水平的降低可能与临床疗效相关.     

双原发乳腺淋巴瘤11例临床分析

目的:探讨双原发乳腺淋巴瘤(bilateral primary breast lymphoma,BPBL)的临床病理特征、诊断、治疗和预后。方法:对本院收治的11例BPBL患者进行临床病理特征、治疗和预后的回顾性分析。结果:11例BPBL患者的年龄为13～63岁,中位年龄34岁;乳腺肿块生长迅速,直径最大者20cm;以双侧乳腺同时发病多见;B症状和血清乳酸脱氢酶水平升高者少见;腋下淋巴结转移和远处转移多见。BPBL病理类型以B细胞来源为主,病理分级以中～高度恶性为主。本组BPBL患者的中位生存期为33个月。化疗对BPBL至关重要。结论:BPBL临床误诊率较高,诊断时必须结合临床病理特征和影像学检查等手段,治疗方式以化疗为主。     

眼眶原发低度恶性粘膜相关淋巴瘤的放射治疗

目的报告6例眼眶原发低度恶性粘膜相关淋巴瘤(MAL Toma)术后放射治疗的结果.复习有关文献,探讨眼眶原发低度恶性粘膜相关淋巴瘤的病理诊断及临床特点和放射治疗的作用.方法对6例患者行术后放射治疗,6MVX线或12～16MeVβ线,单一前野+侧野照射,DT45～55Gy,常规分割.结果放疗后患者无眼损伤及并发症.局部控制良好.结论眼眶低度恶性粘膜相关淋巴瘤是非何杰金氏淋巴瘤的一个亚型,有自身的临床病理特征,应及时确诊,局部放射治疗是有效的治疗方法.