6例幽门螺杆菌阴性的胃粘膜相关组织淋巴瘤临床分析

粘膜相关淋巴组织（mucosa-associated lymphoid tissue, MALT）淋巴瘤是一类来源于B细胞的非霍奇金淋巴瘤，临床经过呈低度恶性，最常发生于胃肠道，胃MALT淋巴瘤约占结外型非霍奇金淋巴瘤的40％。近年研究已证实胃淋巴瘤的发生、发展与幽门螺杆菌（Helicobacter pylori，Hp）感染密切相关，文献报道胃MALT淋巴瘤患者Hp感染率为61％～100％。根除Hp治疗可使约75％的早期胃MALT淋巴瘤患者获得完全缓解，因此成为早期胃MALT淋巴瘤首选治疗，     

原发性胃MALT淋巴瘤临床研究进展

原发性胃淋巴瘤有霍奇金病和非霍奇金淋巴瘤两种类型,临床上后者更多见.1983年Isaacson和Wright提出黏膜相关淋巴组织(MALT)淋巴瘤的概念,并被收编入一个新的分类体系,即修订的欧美淋巴瘤分类(REAL).胃MALT淋巴瘤无论从病因学、病理学、免疫表型和临床归转上,都有其自身特点,1997年WHO将它命名为黏膜相关淋巴组织(MALT)边级区B细胞淋巴瘤.MALT淋巴瘤是结外最常见的淋巴瘤,占非霍奇金淋巴瘤的5%～10%,其中原发于胃的MALT淋巴瘤相对常见,它将是本文讨论的重点[1,2].     

脾边缘区淋巴瘤诊治和预后研究进展

脾边缘区淋巴瘤（SMZL）发病率低,占非霍奇金淋巴瘤1%,临床表现呈惰性过程。本文就脾边缘区淋巴瘤临床病理特征、治疗策略和预后因素方面的研究进展作一综述。     

黏膜相关淋巴样组织型结外边缘区B细胞淋巴瘤的分子细胞遗传学研究进展

黏膜相关淋巴样组织型结外边缘区B细胞淋巴瘤（extranodal marginal zone B—cell lymphoma of mucosa-associated lymphoid tissue，MZL—MALT）是结外最常见的淋巴瘤，近年研究表明慢性感染或自身免疫性疾病所致的直接或间接感染及分子遗传学异常是MZL—MALT发生、发展的基础。     

非霍奇金淋巴瘤临床实践指南(中国版)

2009年(第一版) 边缘带淋巴瘤 边缘区淋巴瘤(marginal zone lymphomas,MZL)是一组异质性疾病,包括结外MZL(MALT淋巴瘤)、淋巴结MZL和脾MZL.MALT淋巴瘤又细分为胃和非胃的淋巴瘤.脾MZL累及脾和骨髓,而结内MZL主要发生在淋巴结,但结外部位也常受累.     

乳腺原发淋巴瘤的诊治

乳腺原发淋巴瘤（ primary breast lymphoma,PBL）临床少见,占所有乳腺恶性肿瘤的0．04%～0．50%,占非霍奇金淋巴瘤的比例不足1%,占所有结外非霍奇金淋巴瘤的1．7%～2．2%,本文回顾性总结了本院收治的3例PBL的临床资料,并结合文献探讨其病理特征、组织学类型、鉴别诊断,治疗与预后。     

B 细胞淋巴瘤之治疗展望

B 细胞淋巴瘤是恶性B 细胞单克隆扩增的一组异质性肿瘤,约占非霍奇金淋巴瘤（non-Hodgkin lymphoma ,NHL ）的85% 。以利妥昔单抗为代表的免疫治疗联合化疗在B 细胞淋巴瘤治疗中获得了巨大成功,患者生存期得到显著改善,但仍有部分复发或病情进展。随着研究和治疗水平的提高,新的靶向药物不断研发和临床应用,自体造血干细胞移植、细胞免疫治疗、放射免疫治疗在血液系统肿瘤中获得较大的进展,给B 细胞淋巴瘤的治疗提供了更多的选择,本文就其最新治疗研究进行综述,并探讨未来治疗的发展方向。      

原发胃肠道淋巴瘤24例

 目的　分析胃肠道淋巴瘤的诊断及治疗方法。方法　对2004年1月至2008年12月诊治的24例原发胃肠道非霍奇金淋巴瘤（NHL）患者的临床资料进行回顾分析。结果　24例中胃淋巴瘤16例（66 %）,其中弥漫大B细胞淋巴瘤（DLBCL）9例,黏膜相关样淋巴组织（MALT）淋巴瘤7例;肠道淋巴瘤8例（33 %）,其中DLBCL 6例,MALT淋巴瘤2例。经胃镜确诊17例,手术确诊7例。DLBCL患者接受CHOP、ECHOP及CHOP样化疗方案,7例经手术确诊的患者有6例术后接受了联合化疗,1例患者自动出院。7例MALT淋巴瘤患者予以CHOP联合抗幽门螺杆菌（HP）治疗,2例予以FMD方案化疗。5例肠道淋巴瘤患者化疗结束后接受累及野放疗。24例患者中3例死亡,1例自动出院失访。目前20例患者生存,其中12例患者完全缓解（CR）,7例患者部分缓解（PR）,1例患者稳定（SD）,化疗有效率79 %,生存率83 %。结论　内窥镜活检目前是胃肠道淋巴瘤诊断最可靠的方法,也是漏诊率、误诊率最低的方法,化疗对胃肠道淋巴瘤的作用已取得共识,具有广泛的适应证,对于已有播散的晚期NHL以及生长迅速的高度侵袭性淋巴瘤患者应首选化疗,胃淋巴瘤手术联合化疗效果并不优于化疗联合放疗。     

不同类型淋巴瘤Survivin的表达及其意义

背景与目的:淋巴瘤的诊断与分型是临床病理诊断的难点。本研究检测抗凋亡基因survivin在不同类型淋巴瘤中的表达,并探讨其对淋巴瘤分型的意义。方法:用免疫组化法检测83例淋巴瘤、5例淋巴结反应性增生石蜡标本中survivin蛋白的表达;同时用RT-PCR检测K562、HL-60、Raji、Jurkat细胞系和以上病例中18例淋巴瘤及2例淋巴结反应性增生新鲜标本中survivinmRNA的表达;对不同类型的淋巴瘤survivin蛋白及mRNA表达进行半定量分析。结果:Survivin蛋白在非霍奇金淋巴瘤(non-Hodgkinslymphoma,NHL)的DLBL(diffuselargeBcelllymphoma)、BL(Burkittlymphoma)、LBL(lymphoblasticlymphoma)中有较高的表达,分别为87.2%(34/39)、100%(2/2)、85.7%(6/7),而在FL(follicularlymphoma)、MALT(extranodalmarginalzoneB-celllymphomaofmucosa-associatedlymphoidtissue)和MZL(marginalzonelymphoma)中表达较低,分别为22.2%、33.3%和40.0%,且多为弱阳性。高表达组(DLBL、BL、LBL)与低表达组(FL、MZL、MALT)之间差异有统计学意义(P<0.01)。并且DLBL中survivin阳性者中位年龄为57岁,明显高于阴性者41岁。霍奇金淋巴瘤(Hodgkinslymphoma,HL)中大部分R-S(Reed-Sternberg)细胞表达survivin蛋白。NHL中survivinmRNA的检测结果与其蛋白水     

MLT1和bcl-6基因表达在胃黏膜相关淋巴组织淋巴瘤鉴别诊断和治疗中的意义

目的研究胃黏膜相关淋巴组织淋巴瘤（MALT淋巴瘤）MLT1、bcl-6基因表达。方法选择已诊断的胃MALT淋巴瘤患者20例作回顾性研究,选择新诊断的患者15例作前瞻性研究。用荧光原位杂交（FISH）法检测石蜡包埋组织MLT1和bcl-6基因表达。结果77.1%胃MALT淋巴瘤表达MLT1基因,分别为Ⅱ～Ⅳ期,化疗后1年内完全缓解率达81.0%。42.9%胃MALT淋巴瘤表达bcl-6基因,均为Ⅲ～Ⅳ期,化疗后1年内完全缓解率达67.0%。Ⅰ期占14.3%,均未检测到MLT1及bcl-6基因表达,选择抗幽门螺杆菌（HP）治疗,1年内完全缓解率达100%。结论FISH法检测胃MALT淋巴瘤患者MLT1和bcl-6基因表达可以为选择个体化治疗方案提供依据。     

Marginal zone lymphoma

Marginal zone lymphomas (MZLs) comprise 3 distinct entities: extranodal MZL of mucosa-associated lymphoid tissue (MALT), splenic MZL, and nodal MZL. Gastric MALT lymphoma is the most common extranodal MZL and often develops as a result of chronic Helicobacter pylori gastritis. Such cases frequently respond to antibiotics directed against H. pylori. Antigen-driven lymphomatous disease can also be seen in the association of Borrelia burgdorferi with MALT lymphoma of the skin, Chlamydia psittaci with MALT lymphoma of the ocular adnexa, Campylobacter jejuni with immunoproliferative disease of the small intestine, and hepatitis C with splenic MZL. This article discusses the pathogenesis and clinical features of MZL and the treatment options available to patients.     

Management of marginal zone lymphomas

Opinion statement Marginal-zone lymphoma (MZL) includes three subtypes depending on the site of lymphoma involvement: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma); splenic MZL; and nodal MZL. Although there is a common cell of origin and similarities concerning a possible chronic antigenic stimulation by microbial pathogens and/or auto-antigens, the clinical presentation is very different with symptoms related to lymphoma location. MALT and splenic MZL present with an indolent disease with good performance status, no B symptoms, and no adverse prognostic factors and are associated with long survival. Patients with nodal MZL present with a shorter progression-free survival. Clinical and biological prognostic factors identified in reported series are heterogeneous. The optimal treatment has yet to be defined for the three subtypes, and current strategies are described in this review.     

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.     

Leukaemic subtype of marginal zone lymphoma: a presentation of three cases and literature review

The recent World Health Organization (WHO) classification recognizes three subtypes of marginal zone lymphoma (MZL): extranodal MZL of mucosa-associated lymphoid tissue (MALT), splenic MZL and nodal MZL. As a group, MZL share morphological and immunophenotypic features similar to that of the marginal zone B-cell in secondary B-follicles, the postulated common cell of origin. There is, however, increasing information about molecular heterogeneity between the types of MZL, suggesting different aetiology and highlighting our current incomplete understanding of this evolving entity. We describe the presentation and clinical course of three patients with MZL who do not fit the currently recognized WHO categories, and best fit the putative category of MZL, leukaemic subtype. We review the current literature on this newly described entity, highlighting the importance of its recognition.     

肺MALT淋巴瘤并腮腺淋巴上皮病1例及文献复习

目的：探讨肺黏膜相关淋巴组织型边缘区B细胞淋巴瘤（MALT-MZL）的临床表现、影像和病理学特点、治疗方法及其与淋巴上皮病（LEL）的关系.方法：报道1例肺MALT淋巴瘤并腮腺淋巴上皮病,对其进行病理组织学观察和免疫组织化检测,并回顾文献.结果：肺MALT淋巴瘤无明显临床表现,影像学特点为边界不清斑片影、结节影、团块影和叶段实变影,可见支气管充气征,缺乏特异性.病理示小淋巴细胞弥漫浸润,支气管、细支气管和肺泡上皮的浸润（淋巴上皮病变）是特征性的,但不特异.免疫组化可显示肿瘤的单克隆性,CD20和CD79a阳性.腮腺LEL是MALT淋巴瘤前期病变,能恶变为MALT淋巴瘤.结论：肺MALT淋巴瘤是一种罕见肿瘤,临床无特异性,确诊靠病理组织学和免疫组化检查,治疗以手术和化疗为主.LEL能恶变为MALT淋巴瘤.     

18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) for staging and follow-up of marginal zone B-cell lymphoma

OBJECTIVE: According to recent reports, nodal marginal zone lymphoma (MZL) appears to be a distinctive lymphoma entity rather than a more advanced stage of extranodal MZL of mucosa-associated lymphoid tissue (MALT). We have therefore retrospectively evaluated all patients diagnosed with nodal or extranodal MZL who have been referred to our unit for imaging using (18)F-fluorodeoxyglucose positron emission tomography ((18)F-FDG-PET). PATIENTS AND METHODS: A total of 21 patients with a diagnosis of MZL upon referral for imaging with (18)F-FDG-PET were identified. Histological reassessment of biopsy specimens confirmed the diagnosis of extranodal MZL of MALT in 14 patients, while a diagnosis of nodal MZL was verified in 6 patients. Lymphoma cell proliferation was assessed immunohistochemically using a Ki-67 antibody. Whole-body (18)F-FDG-PET scans were performed on a GE advanced PET scanner 40 min after intravenous injection of 300-380 MBq (18)F-FDG. RESULTS: None of the patients with extranodal MZL showed focal tracer uptake within verified tumor sites. In contrast, 5 of the 6 patients with nodal MZL showed significant FDG uptake within the affected lymph nodes. These results did not simply reflect the different growth fractions of the two lymphoma entities since the proliferation indices of the two groups did not differ significantly. CONCLUSION:(18)F-FDG-PET visualizes nodal MZL in a high proportion of patients whereas FDG uptake is undetectable in extranodal MZL. Although limited by the small number of patients, this study suggests that imaging with (18)F-FDG-PET might play a potential role in the diagnostic workup of patients with nodal MZL involvement.     

Survival of patients with marginal zone lymphoma

BACKGROUND.

Prognostic factors and outcomes in patients with marginal zone lymphoma (MZL) have been studied in small cohort studies, which may not reflect the population at large.

METHODS.

Clinical characteristics and survival outcomes of adult patients with MZL who were diagnosed between 1995 and 2009 were evaluated using the Surveillance, Epidemiology, and End Results (SEER) database. The authors generated clinical prognostic models for subtypes of MZL and compared survival during the periods of 1995 through 2000, 2001 through 2004, and 2005 through 2009.

RESULTS.

The prognosis was significantly better for patients with mucosa‐associated lymphoid tissue (MALT) lymphoma (5‐year relative survival rate of 88.7%; P < .0001) compared with those with the splenic MZL (SMLZ)or nodal MZL (NMZL) subtypes (5‐year relative survival rates of 79.7% and 76.5%, respectively). There was evidence of improved outcomes in patients with NMZL and MALT lymphomas between 1995 and 2009 (P < .0001), with no difference noted in patients with SMZL (P = .56). Advancing age and the presence of B symptoms had prognostic significance in all MZL subtypes. Male sex and stage of disease were significant only for the NMZL and MALT categories. Survival in patients with MALT lymphomas varied depending on the site of origin, with a worse prognosis noted in those with gastrointestinal and pulmonary locations of origin (5‐year incidence rate of lymphoma‐related death, 9.5%‐14.3%) compared with ocular, cutaneous, and endocrine sites (4.5%‐7.8%; P < .0001).

CONCLUSIONS.

The survival for patients with SMZL is similar to that for those with NMZL, and unlike the NMZL and MALT subtypes, it has not improved over the past decade. The prognosis of patients with MALT lymphoma varies according to the anatomical site of origin. Cancer 2013. © 2012 American Cancer Society.     

Marginal zone B-cell lymphoma: A clinical comparison of nodal and mucosa-associated lymphoid tissue types. Non-Hodgkin's Lymphoma Classification Project.

PURPOSE: In the International Lymphoma Study Group classification of lymphoma, extranodal marginal zone B-cell lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) type is listed as a distinctive entity. However, nodal MZL is listed as a provisional entity because of questions as to whether it is truly a disease or just an advanced stage of MALT-type MZL. To resolve the issue of whether primary nodal MZL without involvement of mucosal sites exists and whether it is clinically different from extranodal MALT-type lymphoma, we compared the clinical features of these two lymphomas. PATIENTS AND METHODS: Five expert hematopathologists reached a consensus diagnosis of MZL in 93 patients. Seventy-three were classified as having MALT-type MZL because of involvement of a mucosal site at the time of diagnosis, and 20 were classified as having nodal MZL because of involvement of lymph nodes without involvement of a mucosal site. RESULTS: A comparison of the clinical features of nodal MZL and MALT-type MZL showed that more patients with nodal MZL presented with advanced-stage disease (71% v 34%; P =. 02), peripheral lymphadenopathy (100% v 8%; P <.001), and para-aortic lymphadenopathy (56% v 14%; P <.001) than those with MALT-type MZL. However, fewer patients with nodal MZL had a large mass (> or = 5 cm) than those with MALT-type MZL (31% v 68%; P =.03). The 5-year overall survival of patients with nodal MZL was lower than that for patients with MALT-type MZL (56% v 81%; P =.09), with a similar result for failure-free survival (28% v 65%; P =.01). Comparisons of patients with International Prognostic Index scores of 0 to 3 showed that those with nodal MZL had lower 5-year overall survival (52% v 88%; P =.025) and failure-free survival (30% v 75%; P =.007) rates than those with MALT-type MZL. CONCLUSION: Nodal MZL seems to be a distinctive disease entity rather than an advanced stage of MALT-type MZL because the clinical presentations and survival outcomes are different in these two types of MZL. Clinically, nodal MZL is similar to other low-grade, node-based B-cell lymphomas, such as follicular and small lymphocytic lymphomas.     

Long term outcomes of gastric mucosa-associated lymphoid tissue lymphoma treated with radiotherapy: A multi-center retrospective cohort study

Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is a rare disease. Radiotherapy remains an important definitive modality. We assessed the long term outcomes of patients with early stage gastric MALT marginal zone lymphoma (MZL) treated with definitive radiotherapy at three institutions in New South Wales, Australia. A retrospective, multi-center study of patients with gastric MALT MZ lymphoma treated with radiotherapy between 1st of March 1999 and 31st of May 2020 was conducted. Eligible patients were: age ≥18 years, treated with curative-intent radiotherapy, pathological diagnosis of MALT MZ lymphoma. There were 33 eligible patients. Complete response (CR) was reported in 30/31 (96.7%) of endoscopically assessed cases. During median follow up of 66.2 months (IQR 22–119 months), estimated 5 and 10 years local relapse free survival were 92.6% (95% CI: 83–100) and 92.6% (95% CI: 83–100); distant relapse free survival 95.8% (95% CI 88.2–100) and 64.7% (95% CI 43.4–96.4); freedom from treatment failure 92.6% (95% CI; 83.1–100) and 62.5% (95% CI; 41.7–93.7), respectively. There were six documented recurrences; one local, four distant, and both in one patient; two cases were high grade recurrences. 5 and 10 years OS were 92.4% and 73.5% respectively. There were no grade 3–5 late toxicities or treatment related deaths. Patients with gastric MALT MZL treated with definitive radiotherapy have excellent outcomes. In long term follow up a significant proportion developed distant low grade disease. Extended follow up should be considered in these patients. Treatment is well tolerated with minimal toxicity. Radiotherapy remains an important modality in the treatment of gastric MALT MZ lymphoma.