Primary extranodal lymphomas of stomach: clinical presentation,diagnostic pitfalls and management

Gastrointestinal lymphoma is the most common form of extranodal lymphoma, accounting for 30%–40% of cases. The most commonly involved site is the stomach (60%–75% of cases), followed by the small bowel, ileum, cecum, colon and rectum. The most common histological subtypes are diffuse large B-cell lymphoma (DLBCL) and marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT). Helicobacter pylori infection has been implicated in the pathogenesis of MALT gastric lymphoma, but its role in gastric diffuse large B-cell non-Hodgkin's lymphoma (NHL) is controversial. The therapeutic approach for patients with gastric NHL has been revised over the last 10 years. Conservative treatment with anthracycline-based chemotherapy alone or in combination with involved-field radiotherapy has replaced gastrectomy as standard therapy in cases with DLBCL. Additionally, MALT lymphomas are mainly treated with antibiotics alone, which can induce lasting remissions in those cases associated with H. pylori infection. Nevertheless, various therapeutic aspects for primary gastric lymphomas are still controversial and several questions remain unanswered. Among others, the role of rituximab, consolidation radiotherapy as well as H. pylori eradication in histological aggressive subtypes warrants better clarification.     

非霍奇金淋巴瘤1012例临床病理分析

目的:了解我院近10年来非霍奇金淋巴瘤(NHL)的发病特点,分析影响NHL预后的相关因素.方法:回顾性分析了近10年来我院收治的1012例NHL患者的临床病理特点,对影响新疆地区NHL生存率及预后的临床病理因素进行分析.结果:1012例NHL中以40～60岁汉族男性发病多见,最主要病理类型依次为弥漫性大B细胞淋巴瘤(DLBCL)346例(34.1%),外周T细胞淋巴瘤(PTCL)185例(18.3%),滤泡淋巴瘤(FL)97例(9.6%),黏膜相关淋巴组织淋巴瘤(MALT)94例(9.3%),NK/T细胞淋巴瘤62例(6.1%),T-淋巴母细胞淋巴瘤(T-LBL)47例(4.6%).结性起病的淋巴瘤619例(61.2%),结外起病的淋巴瘤393例(38.8%).本组维吾尔族女性FL淋巴瘤患者人数比例较汉族女性患者高(P=0.002),汉族男性PTCL患者人数比例高于维吾尔族男性患者(P=0.015).5年总生存率为45.8%.单因素分析显示临床分期,行为状态评分(PS),B症状,年龄,肿块大小,血清乳酸脱氢酶(LDH),结外器官受侵数目及IPI是NHL的预后因素(P＜0.05).多因素分析提示T细胞来源,Ⅲ～Ⅳ临床分期,IPI评分3～5分及LDH增高是NHL独立的预后不良因素(P＜0.05).结论:新疆地区NHL发病以中年多见,结性起病者多于结外起病,B细胞淋巴瘤多于T细胞淋巴瘤.免疫分型、临床分期、IPI、血清LDH水平与NHL预后相关.     

Distribution and clinicopathologic characteristics of non-Hodgkin's lymphoma in India: a study of 935 cases using WHO classification of lymphoid neoplasms (2000)

The frequency of various subtypes of non-Hodgkin's lymphoma (NHL) differs in various regions worldwide. We studied distribution of various subtypes of NHL by using WHO classification of lymphoid neoplasms (2000), immunophenotyping and clinicopathologic characteristics of various histologic subtypes in 935 cases. B- and T-cell NHL constituted 79.3% and 18.8% of cases. Diffuse large B-cell lymphoma (DLBL) was the most common subtype (50.2%). A lower frequency of follicular lymphoma, marginal zone lymphoma and mantle cell lymphoma (MCL) was noted compared to that observed in the developed countries, whereas a lower frequency of peripheral T-cell lymphoma - not otherwise specified (PTCL-NOS) and extranodal NK/T-cell lymphoma was seen compared to that in the other Asian countries. A higher frequency of DLBL and precursor T-lymphoblastic leukemia/lymphoma was noted. Extranodal and bone marrow involvement in MCL and PTCL-NOS was less frequent. Anaplastic variant of DLBL was noted in 21.5% of all DLBLs. Null/T-cell anaplastic large cell lymphoma presented in the older age.     

山东地区520 例淋巴瘤临床病理特点分析

目的:分析山东省地区近3 年淋巴瘤患者的病理类型及临床特点。方法:回顾性分析山东省肿瘤医院及山东省医学科学院附属医院2011年9 月至2014年9 月收治的520 例淋巴瘤患者资料,从性别、发病年龄、病理类型、发病部位等方面进行总结分析。结果:520 例淋巴瘤患者中,男女比例为1. 2 :1,中位年龄51岁,霍奇金淋巴瘤（HL）67例占12.9% ,非霍奇金淋巴（NHL ）453 例占87.1% 。NHL 中75.9% 为B 细胞淋巴瘤,24.1% 为T/NK细胞淋巴瘤。HL以经典型霍奇金淋巴瘤结节硬化型为主,占52.2% ,年龄分布未见双峰性。NHL 最常见类型为弥漫大B 细胞淋巴瘤（DLBCL ）占54.1% ,其构成比高于其他国家和地区（18% ～42.7%）,男女比例为0. 9:1,发病年龄略早（中位年龄54岁）。 HL和NHL 发病部位均以颈部淋巴结多见,且NHL 结外起病多于结内起病。结论:520 例淋巴瘤以DLBCL 最常见,多数亚型以男性发病比例较高,但DLBCL 以女性发病率较高。结外淋巴瘤以鼻腔及胃肠道多见。      

Use of the World Health Organization (WHO) classification of non-Hodgkin's lymphoma in Mumbai, India: a review of 200 consecutive cases by a panel of five expert hematopathologists

This study aims to answer the question whether the World Health Organization (WHO) classification of non-Hodgkin's lymphoma (NHL) can be practised to international standards at the Lymphoma Registry (LR) established at the Tata Memorial Hospital, Mumbai, India. Furthermore, the study aims to identify differences in the distribution of NHL subtypes at this LR (likely to be representative of India) as compared to the rest of the world. A panel of 5 expert hematopathologists from the NHL Classification Project reviewed 200 consecutive NHL cases at the LR in January of 2001. These cases were accrued during August and September, 2000. On all cases, hematoxylin and eosin stains and appropriate immunostains were available for review. The diagnosis made by the host pathologist at the LR (KNN) and the initial diagnosis made by each of the expert hematopathologists was compared with the consensus diagnosis. A consensus diagnosis was made by the 5 experts in 197 cases. The agreement of the host pathologist with the consensus diagnosis was 82% and the agreement of the individual experts with the consensus diagnosis varied from 76-88% (mean 82%). According to the consensus diagnosis, 80% of NHLs were of B-cell type, 18% were of T-cell type, and the immunophenotype could not be determined in the remaining 2% of cases. In conclusion, the WHO classification of NHL was properly utilized at the Lymphoma Registry, Mumbai, India, and geographic differences were noted in the distribution of NHL subtypes at the LR as compared to the rest of the world. Precursor T lymphoblastic leukemia/lymphoma was more common in India (7%) than the rest of the world (1-4%), and indolent B-cell NHLs (29%) were less common than in the West. As compared to China and Japan, peripheral T-cell lymphoma (4.6%), extranodal NK/T cell lymphoma, nasal type (0.5%) and extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma) (2.6%) were less common, but follicular lymphoma (15%) and chronic lymphocytic leukemia/small lymphocytic lymphoma (5%) were more common. This suggests that the distribution of the B-cell and T-cell lymphomas in the Indian population, except for lymphoblastic lymphoma, lies in between the Western world (mainly Caucasian) and the Orientals.     

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.     

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.     

Malignant lymphoma in southern Taiwan according to the revised European-American classification of lymphoid neoplasms

BACKGROUND: The purpose of the current study was to determine the distribution and relative frequency of each subtype of malignant lymphoma in southern Taiwan according to the revised European-American classification of lymphoid neoplasms (REAL). METHODS: The pathology files of a regional hospital in southern Taiwan for 1989-1998 were searched for malignant lymphoma, lymphoproliferative disorder, and Hodgkin disease (HD). The results of light microscopy, immunohistochemistry, and in situ hybridization for Epstein-Barr virus-encoded RNA (EBER) were correlated with clinical findings, and all cases were classified according to REAL. RESULTS: A total of 205 cases were analyzed retrospectively. There were 197 cases (96.1%) of non-Hodgkin lymphoma (NHL) and 8 cases (3. 9%) of HD. Among the 197 NHL cases, 161 (81.7%) were of B-cell lineage and 36 (18.3%) were of T-/natural killer cell lineage. Diffuse large B-cell lymphoma, extranodal marginal zone lymphoma, and follicular lymphoma were the most common B-cell subtypes and represented 47.2%, 19.3%, and 6.1%, respectively, of all NHL cases. Among the 36 cases of T-/natural killer cell lineage, unspecified peripheral T-cell lymphoma (8.6%), T-/natural killer cell lymphoma (angiocentric lymphoma) (4.1%), and anaplastic large cell lymphoma (3.6%) were the most common subtypes. Seven of eight T-/natural killer cell lymphoma cases were positive for EBER. The eight cases of HD were classified as lymphocyte-rich classic (two cases), nodular sclerosis (two cases), and mixed cellularity (four cases) subtypes. Three of these eight cases were positive for EBER. CONCLUSIONS: To the authors' knowledge this study is the first in Taiwan using the REAL classification and it again confirms the different geographic distribution of the various subtypes of malignant lymphoma. The frequency of T-/natural killer cell lineage NHL in Taiwan is higher than that in Western countries but not as high as reported previously.     

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.     

Low incidence of BCL-6 gene alterations for diffuse large B-cell lymphomas in Taiwan Chinese

BACKGROUND: In Western populations, rearrangement of the BCL-6 gene can be identified in 20-40% of patients with diffuse large B-cell lymphoma (DLBCL). Analysis of the BCL-6 gene has revealed the presence of point mutations or small deletions in 70% of DLBCL tumors; however, few studies have investigated BCL-6 gene alteration in patients with non-Hodgkins lymphoma (NHL) of Chinese descent. METHODS: Samples from 135 Taiwanese patients with NHL were examined (28 samples of T-cell NHL and 107 samples of B-cell NHL; 59 samples from patients with DLBCL) for gene rearrangement and mutation of the BCL-6 proto-oncogene using Southern blot analysis and single-strand conformation polymorphism (SSCP) followed by sequence analysis. RESULTS: BCL-6 rearrangement and point mutations were found in 14.8% of patients (n = 20) and in 7.4% of patients (n = 10), respectively. All BCL-6 gene alterations occurred in patients with B-cell NHL, and none occurred in patients with T-cell NHL. Among the 59 patients with DLBCL, BCL-6 gene rearrangements were identified in 10 patients (16.9%), and mutations were identified in 8 patients (13.6%), with the BCL-6 mutation occurring independent of the BCL-6 rearrangement. The incidence of BCL-6 gene rearrangement and mutations in patients with extranodal DLBCL was 9.5% (2 of 21 patients) and 23.8% (5 of 21 patients), respectively. Univariate analysis and multivariate logistic regression found no association between BCL-6 gene alternations and clinical characteristics, including extranodal tumors in patients with DLBCL, and no association between the BCL-6 alterations and prognosis was found. CONCLUSIONS: The incidence of BCL-6 alterations was lower in Taiwanese patients with DLBCL compared with Western populations, and BCL-6 gene alterations showed no prognostic significance in patients with DLBCL.     

Multifocal extranodal non-hodgkin lymphoma: a clinicopathologic study of 37 cases in Greece, a Hellenic Cooperative Oncology Group study

The purpose of this retrospective study was to illustrate the clinicopathological features of patients presenting with multifocal extranodal non-Hodgkin lymphoma (NHL). Among 810 patients with NHL, 37 cases (4.2%) were found to have multiple extranodal involvement (two or more sites). There were 24 men and 13 women, with a median age of 63 years. The majority of these cases (n = 26) had gastric or intestinal (GI) involvement with or without other extranodal sites. Lung along with another extranodal site was relatively common in the present series. Stratification of the 37 cases according to the International Prognostic Index (IPI) showed that 89% of the patients belonged to the high-risk groups. Diffuse large-B-cell lymphoma (DLBCL) accounted for 62%, and mucosa-associated lymphoma tissue (MALT) lymphoma accounted for 27% of all cases. After induction treatment with anthracycline-based regimens, complete remission was achieved in 21 patients (57%), partial remission was achieved in six patients (16%), and seven patients (19%) had no response, while three patients (8%) were nonevaluable. In conclusion, multifocal extranodal NHL is a heterogeneous group of diseases. The majority of them arise at various sites in the GI tract. DLBCL was the most frequent histological subtype followed by MALT lymphoma. Risk group, as defined by the IPI, was predictive of survival.     

Polymorphisms in oxidative stress genes and risk for non-Hodgkin lymphoma

Evidence supporting the contribution of oxidative stress to key pathways in cancer, such as inflammation and DNA damage, continues to mount. We investigated variations within genes mediating oxidative stress to determine whether they alter risk for non-Hodgkin lymphoma (NHL). Thirteen single nucleotide polymorphisms (SNPs) from 10 oxidative stress genes (AKR1A1, AKR1C1, CYBA, GPX, MPO, NOS2A, NOS3, OGG1, PPARG and SOD2) were genotyped in 1172 NHL cases and 982 population-based controls from a USA multicenter case-control study. For NHL and five subtypes (diffuse large B-cell, follicular, marginal zone, small lymphocytic and T-cell), SNP associations were calculated. Odds ratios (OR) and 95% confidence intervals (CI) were adjusted for sex, age (<45, 45-64, 65+ years), race (white, black, other) and study site. Overall, the oxidative stress pathway was associated significantly with the B-cell NHL subtype, diffuse large B-cell lymphoma (DLBCL) (global P-value=0.003). Specifically, for nitric oxide synthase (NOS2A Ser608Leu, rs2297518) Leu/Leu homozygotes, there was a 2-fold risk increase for NHL (OR=2.2, 95% CI=1.1-4.4) (referent=Ser/Ser and Ser/Leu). This risk increase was consistent by cell lineage (B- and T-cell NHL) and pronounced for the two most common subtypes, diffuse large B-cell (OR=3.4, 95% CI=1.5-7.8) and follicular lymphoma (OR=2.6, 95% CI=1.0-6.8). In an analysis of manganese superoxide dismutase (SOD2 Val16Ala, rs1799725) Ala/Ala homozygotes, we observed moderately increased risks for B-cell lymphomas (OR=1.3, 95% CI=1.0-1.6; referent=Val/Val and Val/Ala) that was consistent across the B-cell subtypes. Genetic variations that result in an increased generation of reactive oxygen species appear to increase risk for NHL and its major subtypes, particularly DLBCL. Independent replication of our findings are warranted and further evaluation of oxidative stress in the context of inflammation, DNA repair and the induction of the NF-kappaB pathway may further reveal important clues for lymphomagenesis.     

A Collaborative Nationwide Lymphoma Study in Lebanon: Incidence of Various Subtypes and Analysis of Associations with Viruses

Incidence of various Hodgkin (HL) and non-Hodgkin lymphoma (NHL) subtypes and association with viruses in Lebanon are not known. We undertook a nationwide study of 272 patients diagnosed with lymphoma in 2007. HL comprised 32.7 % (n?=?89) of cases while NHL represented 67.3 % (n?=?183). Consistent with the literature, nodular sclerosis was the most predominant HL subtype (n?=?57/89). Among NHL, B-cell NHL represented 88 % (n?=?161/183), T-cell NHL 9 % (n?=?17/183), whereas in 2.7 % it was not classifiable. The B-cell NHL comprised predominantly diffuse large B-cell lymphoma (46 %) and follicular lymphoma (23 %). 81 cases were reviewed by a panel of pathologists with 87.6 % concordance rate. Serology was negative for hepatitis C in 122 tested cases. HIV was positive in 2 cases. Two adult T-cell leukemia/lymphoma were HTLV-I positive. EBV IgG were positive in 88.5 % of cases. 38 EBV seropositive cases [27 NHL (24 B-cell, 3 T-cell) and 11 HL] were studied for EBV genome expression using EBV-encoded RNA (EBER)-in situ hybridization. EBER expression was positive in 8 (21 %) cases (6 HL, 2 T-cell NHL). The distribution of lymphoma subtypes in Lebanon appears similar to that of Western countries. The high rate of EBV positivity in HL and T-cell lymphoma by EBER deserves further investigation.     

原发乳腺弥漫大B细胞淋巴瘤的诊断与治疗进展

原发乳腺淋巴瘤(PBL)是一种少见的结外淋巴瘤亚型,具有独特的特点.最主要的病理类型为弥漫大B细胞淋巴瘤(DLBCL),其他病理类型比较罕见.文章将以原发乳腺DLBCL为主,阐述其疾病定义、分期,并探讨全身性化疗、局部巩固放疗及利妥昔单抗靶向治疗的地位.     

Primary Extra Nodal Non Hodgkin Lymphoma: A 5 Year Retrospective Analysis

Background and Aim: The incidence of extra nodal non Hodgkin lymphoma (ENL) is rising throughoutthe world. However, data regarding ENL as a group is limited. The aim was to study the epidemiological andhistomorphological trends of primary ENL (pENL) in India. Material and Methods: The biopsy materials fromsixty eight patients with pENL (45 male, 23 female, M:F= 1.9:1), diagnosed over a five year period (2005-2009),were analysed and pathologically reclassified according to the World Health Organization (WHO) classification,2008 criteria. Results: Primary extra nodal non Hodgkin lymphomas constituted 22.0% (68/308) of all nonHodgkin lymphomas (NHL). The mean age at presentation for pENL and primary nodal NHL was 43 years and58 years, respectively with a male predilection (M: F=2:1). Central nervous system (CNS) constituted the mostcommon extranodal site (20/68, 29.5%) followed by gastrointestinal tract (17/68, 25%), and nose/nasopharynx(8/68, 11.8%). Diffuse large B-cell lymphoma (DLBCL, not otherwise specified), extranodal marginal lymphomaof mucosa associated lymphoid tissue (MALT) type, and B cell NHL unclassified (U) were the three most commonhistological types observed. T-cell phenotype was rarely noted (4%). Follicular lymphomas and anaplastic largecell lymphoma, seen among nodal NHL, were absent at extra nodal sites. Majority (41/68, 60%) of the patientswith pENL were immunocompetent and 55% were in stage I-II with favorable prognosis. Conclusion: Centralnervous system was the most common site of ENL, followed by gastrointestinal tract. Majority of pENL occurredin immunocompetent hosts with a favorable prognosis.     

SOHO State of the Art Updates and Next Questions | Challenging Cases in Rare T-Cell Lymphomas

Mature T- and NK-cell neoplasms (MTNKN) collectively represent a rare disorder, representing less than 15% of all non-Hodgkin lymphoma (NHL) cases and qualifying for orphan disease designation by the U.S. Food and Drug Administration (FDA). These consist of 9 families in the fifth revised WHO classification of lymphoid neoplasms, which are made up of over 30 disease subtypes, underscoring the heterogeneity of clinical features, molecular biology, and genetics across this disease group. Moreover, the 5 most common subtypes (peripheral T-cell lymphoma, not otherwise specified; nodal TFH cell lymphoma, angioimmunoblastic type; extranodal NK-cell/T-cell lymphoma; adult T-cell leukemia/lymphoma; and ALK-positive or -negative anaplastic large cell lymphoma) comprise over 75% of MTNKN cases, so other subtypes are exceedingly rare in the context of all NHL diagnoses and consequently often lack consensus on best practices in diagnosis and management. In this review, we discuss the following entities–enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), hepatosplenic T-cell lymphoma (HSTCL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and primary cutaneous ɣδ T-cell lymphoma (PCGD-TCL) – with an emphasis on clinical and diagnostic features and options for management.     

Primary Diffuse Large B-Cell Lymphoma of the Urinary Bladder: Update on a Rare Disease and Potential Diagnostic Pitfalls

Diffuse large B-cell lymphoma (DLBCL) represents the most frequent type of non-Hodgkin lymphoma. Globally, DLBCL is an aggressive disease, requiring an accurate diagnosis and prompt treatment. The diagnosis is often made on biopsy samples of a nodal mass, however, approximately 40% of DLBCL cases arise at extranodal sites. The most common extranodal site is the gastrointestinal tract, however any extranodal area may be primarily involved. Primary urinary bladder lymphoma represents only 0.2% of extranodal non-Hodgkin lymphomas, whereas secondary involvement of the urinary bladder by a systemic lymphoma is a more common event. Despite being rare, DLBCL is considered to represent the predominant primary urinary bladder lymphoma. The majority of cases reported in the bladder belong to the DLBCL, NOS group, and there are only rare cases of EBV-positive DLBCL, NOS. In this review, we summarize the current knowledge on DLBCL primarily occurring in the urinary bladder, with the aim of increasing clinician and pathologist awareness on this aggressive lymphoma rarely arising in the urinary bladder. Additionally, we focus on those entities which should be taken into consideration in the differential diagnosis, highlighting potential diagnostic pitfalls.     

Treatment of T-cell non-hodgkin’ lymphoma

Opinion statement T-cell non-Hodgkin’s lymphoma (NHL) represents approximately 10% to 15% of all lympho-mas in Western countries. Patients with T-cell NHL are often treated similarly to patients with intermediate grade B-cell NHL, although many reports have demonstrated lower overall survival rates in patients with T-cell NHL compared to patients with B-cell NHL. Updated classifications have recognized specific clinical and pathologic T-cell entities, such as peripheral T-cell lymphoma, not otherwise characterized, angioimmunoblastic lymphoma, systemic anaplastic T-cell lymphoma, adult T-cell leukemia/lymphoma, subcutaneous panniculitis-like T-cell lymphoma, hepatosplenic T-cell lymphoma, extranodal natural killer (NK)/T-cell lymphoma nasal type, and enteropathy-type intestinal T-cell lymphoma. Furthermore, these distinct T-cell NHL subtypes often warrant individual-ized diagnostic and therapeutic strategies, such as the associated cytophagic histiocytic panniculitis and hemophagocytic syndrome with subcutaneous panniculitis-like T-cell lymphoma, the chromosomal translocation t(2;5), leading to the nucleophosmin anaplastic lymphoma kinase fusion protein, viral pathogenesis of Epstein-Barr virus, human T-cell lymphotropic virus type-1 associated with extranodal NK/T-cell lymphoma nasal type and adult T-cell leukemia/lymphoma, respectively, and the role of radiation therapy in extra-nodal NK/T-cell lymphoma nasal type. Other active therapeutic agents in T-cell NHL include purine and pyrimidine antimetabolite agents eg, nucleoside analogues and gemcitabine, respectively), denileukin diftitox, and antinucleoside or retinoic acid with interferon-α combination treatment. The exact role of transplantation in patients with T-cell NHL is unknown, but several case series have documented the feasibility of autologous and allogeneic transplant with reported long-term survival rates similar to transplanted B-cell NHL. Identification of relevant proto-oncogenes and tumor suppressor genes involved in the pathogenesis of T-cell NHL, such as the nucleophosmin anaplastic lymphoma kinase fusion protein, p53 and retinoblastoma gene, cyclin-dependent kinase inhibitors, histone deacetylation inhibitors, and infectious etiologies (eg, Epstein-Barr virus and Helicobacter pylori), in addition to their interplay with the various regulatory pathways of cell-cycle progression and apoptosis, represent potential candidates for molecular-based therapy. Prospective multi-institution clinical trials are critically important to determine the most effective treatment regimens that will continue to improve cure rates in these aggressive, yet treatable and often curable, diseases.     

新疆维吾尔自治区乌鲁木齐地区466例非霍奇金淋巴瘤临床病理特征分析

 目的　探讨新疆维吾尔自治区乌鲁木齐地区不同民族非霍奇金淋巴瘤（NHL）的临床病理特点。方法　收集466例NHL标本,复查HE和免疫组织化学染色结果,重新诊断、分型。结果　466例中B细胞性NHL 369例（79.2 %）,T细胞性NHL 97例（20.8 %）;结内193例（41.4 %）,结外273例（58.6 %）;最常见组织学类型为弥漫大B细胞淋巴瘤、小淋巴细胞淋巴瘤／慢性淋巴细胞白血病、黏膜相关淋巴组织结外边缘区B细胞淋巴瘤、NK／T细胞淋巴瘤、外周T细胞淋巴瘤、滤泡性淋巴瘤。维吾尔族T淋巴母细胞淋巴瘤（T-LBL）和间变性大细胞淋巴瘤（ALCL）比例分别为7.5 %（9／120）和4.2 %（5／120）,高于汉族的1.3 %（4／308）和2.3 %（7／308）（χ2＝11.276,P＝0.001;χ2＝1.137,P＝0.286）。而汉族结外NK／T细胞淋巴瘤比例为7.1 %（22／308）,高于维吾尔族的3.3 %（4／120）,差异无统计学意义（χ2＝2.196,P＝0.138）。其余各亚型在不同民族间差异皆无统计学意义（均P＞0.05）。结论　乌鲁木齐地区NHL结外发病高于结内,B细胞性淋巴瘤的发病明显高于我国内地。维吾尔族和汉族B-NHL发病构成在整体上差别不大,但是在T细胞NHL中,维吾尔族T-LBL和ALCL高于汉族,而汉族结外NK／T细胞淋巴瘤高于维吾尔族,这些差异是否与新疆维吾尔自治区的民族与地域差异有关,还需要进一步的研究证实。