非肿瘤性T细胞不完全表达淋巴瘤恶性程度

山西省肿瘤医院病理科副主任王晋芬等同志承担的一项１９９５年回国留学人员科研资助课题研究表明：非肿瘤性Ｔ细胞在肿瘤性Ｂ细胞中的数量并不完全表达淋巴瘤的恶性程度。通过对７８例不同类型淋巴瘤病证实，经免疫双标法定量分析，Ｔ细胞不完全表达淋巴瘤恶性程度，在一些Ｂ细胞淋巴瘤中Ｔ细胞多，恶性程度反而高。该课题探讨了Ｔ细胞在淋巴瘤中的意义，在低度恶性胃ＭＡＬＴ淋巴瘤中肿瘤性Ｂ细胞增生依赖于幽门螺杆菌特异性Ｔ细胞。Ｔ细胞增生可能与患者预后有关。非肿瘤性T细胞不完全表达淋巴瘤恶性程度@霍仕俊     

膀胱原发恶性淋巴瘤的临床病理特点、免疫表型和预后

目的 探讨膀胱原发恶性淋巴瘤的临床、病理组织学特点和免疫表型。方法 回顾性总结7例膀胱恶性淋巴瘤的临床和病理组织学资料，免疫组织化学染色确定免疫表型。结果 6例原发性，1例继发性。7例均为老年患者，女性5例，男性2例；6例原发性结外MALT型边缘区B细胞淋巴瘤4例，弥漫性大B细胞淋巴瘤2例，1例继发是弥漫性大B细胞淋巴瘤。结论 膀胱恶性淋巴瘤好发于60岁左右的老年人，常见的临床表现是血尿和膀胱肿块。常见病理类型是弥漫性大B细胞淋巴瘤和结外MALT型边缘区B细胞淋巴瘤。     

喉非霍奇金淋巴瘤临床病理学观察

目的对喉非霍奇金淋巴瘤（ＬＮＨＬ）这一罕见病变临床病理学特点进行观察，并对其病理诊断、起源组织及与ＥＢＶ感染的关系作一初步探讨。方法复习１８ａ间９例喉ＮＨＬ病理存档资料及临床资料，用免疫组化方法进行病理分型及观察。结果９例均表现为渐进性声嘶伴憋气，６例以呼吸困难急症入院。组织学上多形Ｔ细胞淋巴瘤５例，Ｂ细胞淋巴瘤４例，其中淋巴浆细胞样型和小细胞性各２例。２例Ｂ淋巴瘤作ＥＢＶ免疫组化染色，结果均为阳性。９例中５例确认为喉原发淋巴瘤，２例为上呼吸道多灶病变。结论ＬＮＨＬ中高度恶性的多形Ｔ淋巴瘤超过半数，区分ＬＮＨＬ免疫亚型并探讨其播散方式及与ＥＢＶ感染的关系很有必要。     

58例胃肠道粘膜相关淋巴瘤分型与预后的关系

胃肠道原发恶性淋巴瘤大多数属粘膜相关淋巴瘤（Lymphomasofmucosa－associatedlymphoidtissue，MALT淋巴瘤），少数为其它类型。MALT淋巴瘤可进一步分为低度和高度恶性。我们收集了５８例胃肠道粘膜相关淋巴瘤，根据肿瘤的形态，免疫表型确定肿瘤的恶性程度，其结果与瘤细胞增生性胞核抗原（PCNA）表达和部分患者的预后进行对比分析。１材料与方法１．１材料来源本院病理科１９８４年１月～１９９６年１２月诊断为胃肠道淋巴瘤６５例，根据免疫组化检测的瘤细胞表型以及肿瘤光镜下形态特点，参照１９９３年瑞士第五届国际恶性淋巴瘤…     

膀胱原发恶性淋巴瘤的临床病理特点、免疫表型和预后

目的　探讨膀胱原发恶性淋巴瘤的临床、病理组织学特点和免疫表型。方法　回顾性总结 7例膀胱恶性淋巴瘤的临床和病理组织学资料 ,免疫组织化学染色确定免疫表型。结果　 6例原发性 ,1例继发性。 7例均为老年患者 ,女性 5例 ,男性 2例 ;6例原发性结外MALT型边缘区B细胞淋巴瘤 4例 ,弥漫性大B细胞淋巴瘤 2例 ,1例继发是弥漫性大B细胞淋巴瘤。结论　膀胱恶性淋巴瘤好发于 60岁左右的老年人 ,常见的临床表现是血尿和膀胱肿块。常见病理类型是弥漫性大B细胞淋巴瘤和结外MALT型边缘区B细胞淋巴瘤。     

41例口腔颌面部恶性淋巴瘤的放射治疗

 原发于口腔颌面部的恶性淋巴瘤较少见。Freeman报道1467例结外型ML，仅3例发生在口腔颌面部，吴氏报道40例罕见部位恶性淋巴瘤，口腔颌而部仅5例。     

12例原发甲状腺恶性淋巴瘤的临床病理研究

１２例原发甲状腺恶性淋巴瘤，其中８全炙老年妇女。８例观察到淋巴上皮病损，有７例看到残太腺组织呈淋巴细胞甲状腺炎样变。本组有２例为甲状腺边缘带Ｂ细胞淋巴瘤。免疫组化示１０例为Ｂ细胞性，２例为Ｔ细胞性，分析表明；ＴＬ可能与ＬＴ时的淋巴组织有关，部分ＴＬ可从滤泡旁Ｂ细胞起源。     

非免疫缺陷相关性B细胞淋巴瘤与EB病毒的相关性研究

目的 探讨非免疫缺陷相关性淋巴结内Ｂ细胞淋巴瘤与ＥＢ病毒感染的关系。方法 常规染色进行组织学分类，利用免疫组织化学ＬＳＡＢ法确定肿瘤的免疫表型。采用原位杂交方法检测ＥＢ病毒编码的小核ＲＮＡ（ＥＢＥＲｓ）结果 ４０例淋巴结内Ｂ细胞淋巴瘤中，低度恶性２０列（中心母细胞－中心细胞性１２例，中心细胞性４例，淋巴细胞性４例）高度恶性２０例（中心母细胞性１７例，免疫母细胞性２例，Ｂｕｒｋｉｔｔ样１例）。所有病     

60例非何杰金氏恶性淋巴瘤免疫组织化学研究──附2例Pinkus淋巴瘤报告

本文报告了６０例非何杰金氏恶性淋巴瘤ＭＴ－１、ＭＢ－１ｋ、λ、ＩｇＡ、ＩｇＭ、ＩｇＧ免疫组织化学标记结果。确定Ｂ细胞型２９例，Ｔ细胞型１０例，ｎｏｎ－Ｂ、ｎｏｎ－Ｔ细胞型８例。由于ｎｏｎ－Ｂ、ｎｏｎ－Ｔ细胞构成的肿瘤是一种未分化型肿瘤，细胞没有定向分化，大部分细胞处于幼稚阶段。免疫标记不出细胞属性。本文还发现２例Ｐｉｎｋｕｓ淋巴瘤，细胞核具有Ｔ细胞特点，但ＭＴ－１阴性，ＭＢ－１阳性；ｋ或λ单一阳性，重链两种阳性。证实Ｐｉｎｋｕｓ淋巴瘤是一种核型变异的特殊的Ｂ细胞类型淋巴瘤。     

脾脏原发恶性淋巴瘤（附5例报告）

脾脏原发恶性淋巴瘤（附５例报告）姚怀瑾，郭仁宏恶性淋巴瘤晚期５０％可累及脾脏，但原发于脾的恶性淋巴瘤则较罕见，仅占恶性淋巴瘤总数的１％左右。本文报告了５例脾脏原发性恶性淋巴瘤（以下简称原发脾恶淋）均经手术病理证实，并符合Ｂａｓｇｕｐｔａ（１９６６）提...     

鼻NK/T细胞淋巴瘤中BLU基因启动子区CpG岛甲基化状态研究

 目的 检测鼻NK／T细胞淋巴瘤中抑癌基因BLU启动子区CpG 岛甲基化的状况,并探讨其在鼻NK／T细胞淋巴瘤发生、发展中的作用及与临床病理特征的关系。方法 应用甲基化特异性PCR（methylation-specific PCR, MSP）技术,检测30例鼻NK／T细胞淋巴瘤、10例鼻咽淋巴组织增生中BLU 基因启动子区的甲基化状况;并应用原位杂交方法对30例鼻NK／T细胞淋巴瘤进行EB病毒检测。结果 30例鼻NK／T细胞淋巴瘤中有15例肿瘤组织BLU 基因启动子区CpG 岛发生异常甲基化,甲基化频率为50 %（15／30）,而10例鼻咽淋巴组织增生均无甲基化;EB病毒阳性组BLU 基因的甲基化频率与阴性组对比差异无统计学意义（P＞0.05）;BLU基因启动子区CpG 岛甲基化与临床病理特征之间均无统计学相关性（P＞0.05）。结论 在鼻NK／T细胞淋巴瘤中,抑癌基因BLU启动子区CpG 岛具有很高的甲基化频率,表明其在鼻NK／T细胞淋巴瘤的发生、发展中具有重要作用,并对肿瘤的早期诊断具有重要意义。     

TSLC1和BLU基因在鼻NK／T细胞淋巴瘤中CpG岛甲基化研究

 目的 了解鼻NK／T细胞淋巴瘤中抑癌基因TSLC1和BLU的甲基化状况，并探讨其在鼻NK／T细胞淋巴瘤发生发展中的作用及与临床病理参数之间的关系。方法 应用甲基化特异性PCR（MSP）技术，检测30例鼻NK／T细胞淋巴瘤、10例鼻咽淋巴组织增生中TSLC1和BLU基因启动子区的甲基化状况；应用原位杂交方法对30例鼻NK／T细胞淋巴瘤进行EB病毒检测。结果 30例鼻NK／T细胞淋巴瘤中, TSLC1和BLU基因的甲基化频率分别为83.3 %（25／30）和50 %（15／30），且TSLC1和BLU基因中至少有1个抑癌基因发生甲基化的频率为86.7 %（26／30）；10例鼻咽淋巴组织增生中，2例发生了TSLC1基因甲基化，而BLU 基因全部甲基化阴性而非甲基化阳性。未发现TSLC1和BLU基因CpG 岛甲基化与EB病毒感染有关。TSLC1和BLU基因启动子区CpG岛甲基化与临床病理特征之间均无统计学相关性（P＞0.05）。结论 30例鼻NK／T细胞淋巴瘤中多基因的启动子甲基化是一种普遍的事件。两种抑癌基因启动子区CpG 岛均具有很高的甲基化频率，表明其在鼻NK／T细胞淋巴瘤的发生发展中具有重要作用，可能对肿瘤的早期诊断及预后评估具有重要意义。     

Malignant lymphoma in patients with rheumatic diseases other than Sjogren's syndrome: a clinicopathologic study of five cases and a review of the Japanese literature

We conducted clinicopathologic and immunohistochemical analysis of five patients with malignant lymphoma complicating rheumatic diseases other than Sjogren's syndrome, and reviewed 26 cases of similar lesions reported in the Japanese literature over a 17-year period. All five patients were women ranging in age from 31 to 74 years (mean 55 years). Two of them fulfilled the diagnostic criteria for systemic lupus erythematosus, two for dermatomyositis and one for progressive systemic sclerosis. The use of immunosuppressive drugs before the onset of malignant lymphoma was recorded in four patients. All the biopsied or resected specimens showed non-Hodgkin's lymphoma of B-cell phenotype. Three were nodal in origin (one diffuse mixed, one diffuse large cell and one immunoblastic) and two were extranodal (one low-grade B-cell lymphoma of mucosa-associated lymphoid tissue and one diffuse large cell). In three of four cases examined, Epstein-Barr virus-encoded small RNAs were identified in a small to large number of the lymphoma cells by in situ hybridization. Our study showed that the clinicopathological features of malignant lymphomas complicating rheumatic disease in Japan were similar to those in England and the USA. Furthermore, our findings suggested no evidence for a causative association between iatrogenic immunosuppression due to methotrexate therapy and the development of EBV-related lymphoid neoplasms.      

Distribution and prognosis of WHO lymphoma subtypes in Taiwan reveals a low incidence of germinal-center derived tumors

To assess the distribution of lymphomas in Taiwan according to the WHO (World Health Organization) classification, 175 recently diagnosed cases of malignant lymphomas were studied and the clinicopathologic data were analyzed. B-cell lymphomas accounted for 57.1% of cases, T-cell lymphomas 38.9%, and Hodgkin's lymphoma 4%. Extranodal lymphomas predominated (55.4%). The most common subtype of B-cell lymphoma was diffuse large B-cell lymphoma (33.1%). All tumor types believed to be derived from germinal center (GC) B-cells including follicular lymphoma (4.6%), Burkitt lymphoma (1.7%), Hodgkin lymphoma (4.0%), and GC-like diffuse large B-cell lymphoma (as defined by combined expression of bcl-6 and CD10) were rather uncommon as compared to frequencies seen in series from Western countries. The common T-cell lymphomas included nasal and extranasal NK/T cell lymphoma (7.4%), mycosis fungoides (7.4%), and unspecified peripheral T-cell lymphoma (6.9%). Adult T-cell leukemia/lymphoma was very uncommon and accounts for only 0.6%. The proportional increase in T-cell lymphomas that were unrelated to type I human T-cell lymphotropic virus (HTLV-1) may be linked to differential Epstein-Barr virus (EBV) oncogenesis. The survival data revealed that mantle cell lymphoma, NK/T-cell lymphoma, unspecified peripheral T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma had an aggressive course. Our results confirm the utility of the WHO classification scheme for prognostic stratification and further highlight the distinctive distribution pattern of malignant lymphoma in Taiwan including the higher relative incidence of T cell lymphomas and the rarity of germinal center-derived B-cell tumors.     

恶性肉芽肿与中线淋巴瘤的临床观察

目的探讨恶性肉芽肿与中线淋巴瘤两种疾病的关系。方法自１９８９年１２月至１９９５年３月共收治恶性肉芽肿及中线淋巴瘤各２３例。两者在发病年龄、部位及首发症状相似。前者有７３．９％单用放射治疗,后者有６９．５％用放射治疗和化疗联合。病理类型前者为炎性坏死性肉芽组织,后者均为非霍奇金淋巴瘤（Ｔ细胞性１３例,Ｂ细胞性１例,未分型９例）。结果完全缓解率分别为８２．６％和６５．２％,２年生存率分别为８２．６％和７３．９％。结论两者不应视为同一疾病。     

Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx: a clinicopathologic study. X. Malignant lymphomas.

In our series of 256 nonepithelial tumors involving the nasal cavity, paranasal sinuses and nasopharynx, 21 were apparently primary malignant lymphomas, including 17 ordinary lymphomas and 4 cases of "midline malignant reticulosis." Of the 15 patients who had ordinary lymphomas and had adequate follow-up, 8 died of lymphoma, 4 were living with disseminated disease, 1 died of other causes with persistent lymphoma and only 2 (13%) had no evidence of recurrence at 8 and 9 years after diagnosis. The tumor was controlled in its primary site by radiotherapy in 13 of 14 patients; however, all but 2 of these patients eventually developed disseminated disease. Of the 3 patients who had midline malignant reticulosis (MMR) and had adequate follow-up, all died of disease. MMR represents an unusual variant of malignant lymphoma and often produces the clinical picture of lethal midline granuloma.     

Body cavity-based presentation of natural killer cell lymphoma

We describe an unusual case of a 31-year-old Mexican woman who presented with pleural and peritoneal effusions involved by Epstein-Barr virus-positive non-Hodgkin's lymphoma of natural killer (NK)-cell lineage. The patient had no symptoms that could be related to her nasal region, and physical examination and radiologic studies showed no evidence of lymphadenopathy, organomegaly, or other extranodal masses. Thus, this case clinically mimicked body cavity-based lymphoma. Extranodal NK/T-cell lymphoma of nasal type is the current designation for these neoplasms in the recently proposed World Health Organization classification of lymphoid neoplasms. These tumors previously have been referred to many other names, including lethal midline granuloma, midline malignant reticulosis, polymorphic reticulosis, angiocentric immunoproliferative lesion, and angiocentric lymphoma. Nasal-type NK/T-cell lymphomas typically involve the nasal region, but may involve other extranodal sites, such as skin and gastrointestinal tract. The malignant cytologic features and the presence of azurophilic granules within the cell cytoplasm observed in Wright-Giemsa-stained cytocentrifuge preparations led to immunophenotypic and molecular genetic studies that were essential in establishing the correct diagnosis. As demonstrated in the case reported, extranodal NK/T-cell lymphomas of nasal-type can be clinically aggressive and may be associated with paraneoplastic phenomena.     

Emerging pathways in the development of AIDS-related lymphomas

The clinicopathological range of AIDS-related non-Hodgkin lymphomas (NHLs) includes systemic lymphomas, primary central-nervous-system lymphomas, primary effusion lymphoma, and plasmablastic lymphoma of the oral cavity. Most AIDS-related NHLs belong to one of three categories of high-grade B-cell lymphomas: Burkitt's lymphoma, centroblastic lymphoma, and immunoblastic lymphoma. The pathological heterogeneity of AIDS-related NHL reflects the heterogeneity of their associated molecular lesions. In AIDS-related Burkitt's lymphoma, the molecular lesions involve activation of c-MYC, inactivation of p53, and infection with Epstein-Barr virus (EBV). AIDS-related immunoblastic lymphomas infected with EBV are characterised by frequent expression of latent membrane protein 1-an EBV oncoprotein. The biological heterogeneity of AIDS-related NHLs is highlighted by their histogenetic differences; AIDS-related NHLs are related to distinct B-cell subgroups (eg, germinal-centre or post-germinal-centre B cells). The phenotypic pattern of AIDS-related Burkitt's lymphomas and systemic AIDS-related centroblastic lymphomas closely reflects that of B cells in germinal centres. Conversely, the phenotype of AIDS-related immunoblastic lymphomas and AIDS-related primary effusion lymphomas reflects post-germinal-centre B cells in all cases. Despite their clinicopathological, genetic, and phenotypic heterogeneity, most lymphomas in patients with AIDS carry somatic mutations of immunoglobulin and BCL-6 genes. However, the somatic hypermutation mechanism functions aberrantly in a significant proportion of AIDS-related NHLs, causing the mutation of many genes, and possibly favouring chromosomal translocation, which may be a powerful contributor to malignant transformation. New molecular and virological evidence of such pathways and a greater knowledge of other biological features of AIDS-related NHLs may lead to new targets for pathogenetically and biologically oriented therapies.     

Clinicopathological analysis of 17 primary cutaneous T‐cell lymphoma of the γδ phenotype from Japan

Primary cutaneous γδ T‐cell lymphoma (PCGD‐TCL) is an aggressive lymphoma consisting of clonal proliferation of mature activated γδ T‐cells of a cytotoxic phenotype. Because primary cutaneous γδ T‐cell lymphoma is a rare disease, there are few clinicopathological studies. In addition, T‐cell receptor (TCR) γδ cells are typically immunostained in frozen sections or determined by TCRβ negativity. We retrospectively analyzed 17 primary cutaneous T‐cell lymphomas of the γδ phenotype (CTCL‐γδ) in a clinicopathological and molecular study using paraffin‐embedded sections. Among 17 patients, 11 had CTCL‐γδ without subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) features and six had CTCL‐γδ with SPTCL features. Immunophenotypically, some significant differences were found in CD8 and CD56 positivity between our patient series of CTCL‐γδ patients with SPTCL features and SPTCL‐γδ patients described in the previous literature. A univariate analysis of 17 CTCL‐γδ patients showed that being more than 60 years old, presence of visceral organ involvement, and small‐to‐medium cell size were poor prognostic factors. In addition, the 5‐year overall survival rate was 42.4% for the CTCL‐γδ patients without SPTCL features and 80.0% for those with SPTCL features. Consequently, there was a strikingly significant difference in overall survival among SPTCL, CTCL‐γδ with SPTCL features and CTCL‐γδ without SPTCL features (P = 0.0005). Our data suggests that an indolent subgroup may exist in CTCL‐γδ. Studies on more cases, including those from other countries, are warranted to delineate the clinicopathological features and the significance in these rare lymphomas.     

Practical Utility of the Revised European-American Classification of Lymphoid Neoplasms for Japanese Non-Hodgkin's Lymphomas

A clinicopathological study of 515 non-Hodgkin's lymphoma (NHL) cases was performed using the revised European-American classification of lymphoid neoplasms (REAL classification) in an HTLV1-nonendemic area of Japan. The following characteristics were revealed: 1) frequency of extranodal lymphomas was high (59%) with 79% B-cell lymphomas in this series, while the overall ratio of B:T/NK lineage was 3.7:1; 2) the most common type was the diffuse large B-cell lymphoma (46%), follicle center lymphomas occurred at an incidence lower (15%) than that in European and American populations, and marginal zone B-cell lymphomas accounted for as much as 12%; 3) peripheral T-cell lymphomas were common (19%), with the unspecified type predominant (11%), while adult T-cell lymphomas were present at a level equivalent to that among European and American patients (1%). Clear segregation of survival curves was rated according to cell lineage and B-cell lymphomas had a better prognosis than T/NK-cell lymphomas. Furthermore, new subtypes in the REAL classification, such as marginal zone B-cell and mantle cell lymphomas, exhibited distinct curves. Taken altogether, the REAL classification demonstrated advantages for assessment of Japanese NHL cases.