原发性中枢神经系统淋巴瘤临床分析

目的:探讨原发性中枢神经系统淋巴瘤(PCNSL)的临床特征.方法:回顾分析10例免疫功能正常的PCNSL患者的临床资料.结果:临床表现为颅内压增高及病灶所致的局部表现.病灶单发7例,多发3例,全经手术切除病灶,病理示B细胞型非霍奇金淋巴瘤.6例术后未行放化疗出院,失访1例,随访5例,平均生存4个月.1例CHOP方案化疗1周期,生存19个月.3例行大剂量甲氨蝶呤化疗+放疗;1例生存13个月,2例至今生存,平均生存24个月,复查无异常.结论:PCNSL预后不良,单纯手术不能延长患者生存期,手术结合放化疗是目前临床首选的治疗方案.     

骨髓红系细胞凋亡、增殖及促红细胞生成素受体表达在恶性淋巴瘤贫血中的意义

目的　探讨骨髓红系凋亡、增殖状态及促红细胞生成素受体 (EPOR)表达在恶性淋巴瘤贫血发病机理中的意义。方法　以转铁蛋白受体 (CD71)和血型糖蛋白A(GPA)作为红系标志 ,采用流式细胞术检测骨髓红系细胞的凋亡率、增殖率及EPOR的表达。结果　恶性淋巴瘤骨髓红系CD+ 71细胞和GPA+ 细胞凋亡率明显高于正常对照组 ,差异具有非常显著意义 (4 4 .2 8± 2 4.5 2对 11.0 7± 7.16,P <0 .0 0 1和 47.79± 2 8.3 7对 11.3 5± 5 .98,P <0 .0 0 1) ;增殖率也明显高于正常对照组 (4 9.63± 3 1.68对 2 8.47± 13 .63 ,P <0 .0 5和 5 0 .63± 2 8.74对 2 9.5 9± 9.81,P <0 .0 5 ) ;EPOR表达的平均荧光强度 (MFI)低于正常对照组 ,但差异无显著意义 (P >0 .0 5 )。CD+ 71细胞和GPA+ 细胞凋亡率与血红蛋白 (Hb)水平呈一定的负相关 (r =-0 .60 0 9,P <0 .0 5和r =-0 .612 2 ,P <0 .0 5 ) ;EPOR表达与Hb水平无明显相关。结论　红系细胞凋亡率增高可能同恶性淋巴瘤贫血的发病机制有关 ,红系的明显增殖可能是对凋亡的反馈调节。EPOR表达水平降低可能不是恶性淋巴瘤贫血发病的普遍机制。     

结肠原发性恶性淋巴瘤26例诊治分析

［摘要］　目的　探讨结肠原发性恶性淋巴瘤的临床特点、诊断及治疗。方法　回顾性分析26例结肠原发性恶性淋巴瘤的临床资料。结果　26例主要表现为腹痛、腹胀，均为非霍奇金淋巴瘤。内镜活检确诊率为38.46%。接受手术及化疗的患者病情均有不同程度好转。结论　结肠原发性恶性淋巴瘤的临床表现缺乏特异性，误诊率高，手术加化疗治疗本病疗效较好。     

非霍奇金淋巴瘤患者微量白蛋白尿的临床意义

目的 :探讨非霍奇金淋巴瘤 (NHL)患者的尿蛋白排泄率 (UAE)水平及其临床应用价值。方法 :采用放射免疫分析方法测定 48例NHL患者初诊时 2 4h的UAE水平 ,对治疗前UAE≥ 2 0 μg/min的患者在治疗达到缓解后进行复查 ,并与发病年龄 ,血清LDH ,结外受累 ,巨大包块 ,分期等重要预后因素进行相关分析。结果 :UAE的中位数是 15 .0 μg/min ,微白蛋白尿 (UAE≥ 2 0 μg/min)的发生率是39.6 %。UAE的水平与疾病分期、血清LDH水平相关 ,治疗后UAE的中位数与治疗前相比有明显降低 (P <0 .0 1)。结论 :NHL患者UAE水平与肿瘤负荷及患者预后相关。     

胃弥漫性大B细胞淋巴瘤一例

<正>1病例介绍患者,男,72岁,主因"便秘2月余,伴腹胀、食欲不振1月"入院。入院前2月无明显诱因出现排便不畅,4~5 d一行,大便干稀不调。无脓血便。入院前1月出现腹胀、食欲不振,遂就诊于我院。入院时患者大便6 d未结,伴腹胀、食欲不振,无腹痛、恶心、呕吐。寐安,小便可。查体:体温38.3℃,脉搏84次/min,呼吸18次/min,血压140/70 mm Hg。     

成人原发性胃肠道Burkitt样淋巴瘤的诊治

目的:探讨成人原发性胃肠道Burkitt样淋巴瘤的流行病学、临床病理特点、诊断和治疗.方法:利用韩国延世大学图书馆平台在Medline/Pub Med、Web of Science、Directory of Open Access Journals(DOAJ)、Springer Link等15家英文文献数据库和中国知网中文期刊全文数据库中检索1995年以来发表的符合纳入条件的相关文献,共获得来自6篇文献的6例成人原发性胃肠道Burkitt样淋巴瘤的患者资料.结果:成人原发性胃肠道Burkitt样淋巴瘤主要分布在东亚的日本和中国(4例,66.7%);6例患者均为男性,平均年龄57.6岁;2例发生于回盲部,1例发生于胃,1例发生于结肠肝曲,1例发生于末端回肠,还有1例在胃和小肠同时发生;因发现腹部肿块就诊者4例,伴随B症状:体质量减轻4例;乏力2例;所有患者均行消化道内镜检查,行计算机断层扫描(computed tomography,CT)检查者5例,行B超检查者3例;行手术治疗者4例,行联合化疗者5例,行手术+术后化疗者3例;1例患者在1年内死亡;最长1例患者随访5年仍存活;6例患者中行CD20检测者4例,均为阳性;行C D10检测者6例,4例为阳性;行K i-67检测者3例,均为阳性;行B淋巴细胞瘤-2(B-cell lymphoma-2,Bcl-2)检测者3例,均为阴性;行CD23检测者2例,均为阴性;行CD5检测者2例,均为阴性.结论:成人原发性胃肠道Burkitt样淋巴瘤临床罕见,属于高度恶性的非霍奇金淋巴瘤,进展快,死亡率高.其临床表现缺乏特异性,在进行临床干预治疗之前,建议行全面的检查以明确诊断,最终诊断仍依赖于细针穿刺活组织检查或手术切除病理检查.治疗上应在诊断明确后尽早实施外科手术及术后的多药联合辅助化疗.临床医师应该提高对本病的认识,重视该病的临床诊治,以改善患者的预后.     

淋巴细胞性甲状腺炎转化为非霍奇金淋巴瘤1例

患者男,54岁.因右颈前肿物伴颜面潮红2年,于1992年9月10日入我院.1983年无意中发现右颈前部有一5cm×8cm肿块,无不适感,无发热、消瘦、多汗等症状.甲状腺B超疑为甲状腺癌,在某医院行右侧甲状腺大部及椎体叶切除术.病理检查结果:右侧淋巴细胞性甲状腺炎.1990年发现原右颈前部肿块上方又出现1肿块,无红肿痛感.颜面发热,潮红状.未治疗.1992年6月,开始声音嘶哑,喘气,心悸,咳嗽.检查:一般情况可.右颈前部可扪及3cm×4cm肿块,质地中等,光滑,边缘清楚,无红、肿、痛.颜面潮红.肝脾未触及.血象:Hb130g/L,WBC7.O×10~9/L,中性0.60,淋巴0.30,嗜酸性0.02,单核,0.08BPC130×10~9/L;血沉12mm/h;尿常规、肝肾功能正常,血电解质正常,血T_3、T_4正常,胸部CT提示中上纵隔淋巴结肿大.颈前肿块活检,发现瘤性小淋巴细胞弥漫增生,排列稠密,肿瘤细胞比正常淋巴细胞大,大小较一致,核小而圆,罕见核裂,染色质浓染,胞浆极少.病理诊断:非霍奇金淋巴瘤(弥漫性小细胞型;B细胞来源),骨髓涂片检查:未见淋巴瘤细胞浸润.经采用COPP方案,2个疗程化疗后,颜面潮红、心悸、喘气缓解,发音正常.复查胸部CT:中上纵隔淋巴结明显缩小.现继续用COA(阿霉素)方案化疗.甲状腺恶性淋巴瘤发病率低.有人认为此恶性淋巴瘤可能来源于慢性淋巴细胞性甲状腺     

Primary non-Hodgkin lymphomas in the small and large intestine: clinicopathological characteristics and management of 40 patients

To investigate the clinicopathological characteristics and optimal treatment modalities of primary non-Hodgkin lymphoma (NHL) in the small and large intestine. Forty patients with primary NHL in the small and large intestine were studied retrospectively. All cases were reclassified according to the World Health Organization (WHO) classification of lymphoma in 2001. Fourteen patients had primary disease in the small intestine, which were all of B-cell origin with diffuse large B-cell lymphoma (DLBCL) diagnosed in 5 of 14 (35.7%) patients and mucosa-associated lymphoid tissue (MALT) lymphoma in 8 of 14 (57.1%) patients. Ileum was the most commonly involved site (8 of 14 patients, 57.1%), followed by jejunum (2 of 14 patients, 14.3%) and duodenum (1 of 14 patients, 7.1%). Twenty-five patients had primary colorectal lymphoma, with B-cell origin accounting for 92.0% and T-cell origin for 8.0% of these patients. The ileocaecal region has the highest involved rate (13 of 25 patients, 52.0%), followed by colon (7 of 25 patients, 28.0%) and rectum (3 of 25 patients, 12.0%). Compared with surgery alone, post-operation chemotherapy or chemoradiotherapy can significantly improve DLBCL patients’ event-free survival (EFS). However, no post-operation treatment modality can improve OS or EFS for patients with MALT lymphoma. B-cell lymphoma is the most common pathological type of intestinal lymphomas. Chemotherapy-containing treatment modality is an effective way to improve intestinal lymphoma patients’ EFS, especially for those with DLBCL subtype.     

不同部位原发性胃肠道淋巴瘤的临床特点

目的 研究不同部位原发性胃肠道淋巴瘤(PGIL)的临床特点,以提高对该疾病的诊断水平.方法 回顾性分析武汉地区8家医院1999年1月至2007年6月经病理确诊的PGIL患者,共有202例资料完整的病例用于最后统计,按部位分为胃、小肠、大肠淋巴瘤三组,并比较三组间的临床特点.结果 PGIL发生于胃113例(56.0%)、小肠37例(18.3%)、大肠52例(25.7%);男130例(64.4%),女72例(35.6%),均以男性发病率为高.胃淋巴瘤组比小肠淋巴瘤组的病程长(3个月比1个月,P=0.013).三组PGIL均以腹痛、贫血发生率最高,其中伴有贫血症状者大多为轻度(57.9%).PGIL临床分期均以Ⅰ E期、ⅡE期为主,占71.3%;与胃淋巴瘤组比较,大肠淋巴瘤组的临床分期相对较重(P=0.014);PGIL主要病理类型为黏膜相关淋巴组织(MALT)淋巴瘤、弥漫大B细胞淋巴瘤、T细胞淋巴瘤.胃淋巴瘤组以低度恶性MALT淋巴瘤多见(56.9%),而小肠淋巴瘤组以T细胞淋巴瘤多见(34.4%),大肠淋巴瘤组以高恶性B细胞淋巴瘤多见(51.1%).PGIL病灶类型以隆起肿块型、溃疡型为主,但与胃淋巴瘤组相比,大肠淋巴瘤组以隆起肿块型居多而溃疡型少见(P=0.000).胃、小肠、大肠淋巴瘤组经内镜并活检的确诊率分别为58.7%(61/104)、25.0%(4/16)、48.2%(13/27).结论 不同部位PGIL的临床症状、病理分型,临床分期、病灶类型,内镜检出率各有特点,这些差异有助于临床医师对PGIL的认识及诊断.     

Clinicopathological analysis of T-cell lymphoma in Taiwan according to WHO classification: high incidence of enteropathy-type intestinal T-cell lymphoma

OBJECTIVES: The clinicopathological characteristics of malignant lymphomas vary according to geography, especially for the T-cell lymphoma (TCL). The aim of this study is to demonstrate the incidence and clinicopathological characteristics of TCL in Taiwan according to WHO classification. METHODS: Archival tissue from 600 malignant lymphomas during the period of 1995-2002 was retrieved, there were 74 cases diagnosed with TCL. Hematoxylin and eosin slides stained and other special studies were reviewed. All cases were reclassified according to the WHO classification. Clinical data, including age, gender, clinical staging, and follow-up, were scrutinized. RESULTS: There were 52 males and 22 females with male predilectation. The incidence of TCL in malignant lymphoma was 12.3%. The most prevalent histologic subtype was peripheral T-cell lymphoma (PTCL), followed by nasal T-cell/Natural killer- (T-/NK-) cell lymphoma, T-lymphoblastic lymphoma (LBL), anaplastic large cell lymphoma, and enteropathy-type intestinal lymphoma (ETCL). Clinically, 39 cases (49%) had higher clinical stage (III/IV). Overall 2-year and 5-year survival rate was 51.4% and 34.7%, respectively. It was noteworthy that ETCL had high incidence rate (8.1%) and high mortality. There was significant difference in survival rates in respect of clinical stages and histological subtypes. CONCLUSIONS: TCL is relatively less frequent in Taiwan, compared to other countries in Asian and western countries. The most common histological subtypes are PTCL, unspecified and T-/NK-cell lymphoma. Five-year survival rate is 34.7%. ETCL has higher incidence rate in Taiwan. The clinical stage and histological subtypes are prognostic parameters in determining the survival rates.     

Frequencies of non-Hodgkin’s lymphoma subtypes in Kuwait: comparisons between different ethnic groups

There is a wide variation in the prevalence of various subtypes of non-Hodgkin’s lymphoma worldwide. The aim of this study was to determine the relative frequency of different subtypes of non-Hodgkin’s lymphoma in Kuwait based on the Revised European–American Lymphoma (REAL) classification. From 1998 to 2006, 738 subjects were included that were registered with non-Hodgkin’s lymphoma in the population-based cancer registry at the Kuwait Cancer Control Center. Expert pathologists reviewed histological slides from all subjects. We performed detailed immunohistochemical studies and classified subjects based on the REAL classification. The prevalence of different types of non-Hodgkin's lymphoma was determined based on age, sex, site of disease, and ethnicity. Ethnicity groups comprised Kuwaiti Arabs, non Kuwaiti Arabs, Asians, and others. The prevalence of B- and T-cell lymphomas was 81.8% and 14.2%, respectively. The most common age group was 41–60 years old. The three most common subtypes in Kuwaiti Arabs were diffuse large B-cell lymphoma (46.5%), follicular lymphoma (15.5%), and mycosis fungoides (9.3%). In non-Kuwaiti Arabs, the most common subtypes were diffuse large B-cell lymphoma (48%), B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia (15.8%), and follicular lymphoma (12.7%). Overall, non-Kuwaiti Arabs exhibited the highest prevalence (59%), and 54% of all cases had extranodal presentation. Compared to the Western world, Kuwait had a lower prevalence of follicular lymphoma, a higher prevalence of diffuse large B-cell lymphoma and extranodal presentation, and a high frequency of mycosis fungoides. Compared to other parts of Asia, Kuwait had a lower frequency of peripheral T-cell lymphomas.     

Secondary T-cell lymphoma in the setting of nodular lymphocyte predominance Hodgkin's disease.

Lymphocyte predominance Hodgkin's disease (LPHD) can be histologically subdivided into a nodular and diffuse variety. The two subtypes differ in immunophenotypic characteristics but have a similar long-term clinical outcome. Nodular LPHD has immunophenotypic and histological characteristics suggestive of a B-cell derived neoplastic process. Nodular LPHD is associated with an increased risk of secondary large cell lymphoma (LCL). Gene rearrangement studies in some of these cases have revealed a B-cell clonal process, further supporting the association between nodular LPHD and the B-cell system. In addition, it has been suggested that the apparent secondary LCL, at least in some cases, may represent a histologic progression of nodular LPHD. We report a unique case of T-cell lymphoma, confirmed by T-cell receptor gene rearrangement studies, which developed in the setting of nodular LPHD. Our observation demonstrates that the association of nodular LPHD and LCL is complex and that LCL developing in the context of nodular LPHD may be an independent secondary process sometimes involving T-cell lymphomas.     

Infiltration of cervical lymph nodes by B- and T-cell non-Hodgkin's lymphoma and Hodgkin's lymphoma: preliminary ultrasonic findings.

Ultrasonography has been used in the evaluation of enlarged lymph nodes in patients with malignant lymphoma, which comprises a variety of histopathological subtypes. We assessed ultrasonic findings of infiltration in cervical lymph nodes of 47 patients with previously untreated malignant lymphoma and correlated them with histopathological subtypes. Common findings among various histopathological subtypes were hypoechoic masses, spot and/or line echoes, involvement of multiple nodes, and posterior enhancement. In addition, each histopathological subtype showed characteristic findings: Hodgkin's disease had a contour with a lobular pattern (9/10 cases), septum-like echo (9/10 cases), and tubular structure (9/10 cases) in the internal echo, reflecting histopathologically fibrous connective tissue surrounding lymph nodes and small vessels. In B-cell lymphoma, there was characteristically a tendency for lymph node fusion (19/28 cases) and spot and/or line echoes (17/28 cases), reflecting histopathological replacement and destruction of the structure of lymph node and capsule by proliferation of lymphocytes. These findings were strengthened in diffuse large B-cell lymphoma. T-cell lymphoma was distinguished from other types of lymphoma by the presence of a hilum-like echo (6/9 cases), reflecting histopathologically prominent vascularization in lymph nodes accompanied by a dilated hilum area. In summary, this preliminary study suggests that ultrasonic findings of infiltration of cervical lymph nodes in major subtypes of malignant lymphoma could reflect characteristic histopathological structures, therefore providing information helpful for differentiating them.     

Presence of B-cell clones in T-cell lymphoma

Objectives: The presence of B-cell clones in 76 cases with peripheral T-cell lymphoma (PTCL) and precursor T-lymphoblastic lymphoma (T-LBL) and its correlation with Epstein-Barr virus (EBV) was studied. Methods: DNA was extracted from paraffin sections and/or fresh-frozen samples and then used for clonality analysis using a modified BIOMED-2 polymerase chain reaction (PCR)-based method. Results: T- and B-cell clones were detected in 59 (77.6%) and 14 (18.4%) of 76 patients, respectively: 90% and 30% of cases with PTCL, not otherwise specified, 76.4% and 17.6% of cases with angioimmunoblastic T-cell lymphoma, 77% and 7.6% of cases with adult T-cell lymphoma, 50% and 0% of cases with anaplastic large T-cell lymphoma, 62.5% and 12.5% of cases with T-LBL, and 50% and 0% of cases with intestinal T-cell lymphoma, respectively. Histological and immunohistochemical analyses revealed the presence of large B cells in lesional tissues, which were occasionally monoclonal. The presence of B-cell clones was highly associated with EBV positivity, as revealed by in situ hybridization. In two cases that were evaluated by serial histological and molecular examination, EBV-positive cells persisted in one and disappeared in the other. Conclusions: These findings suggest a role for EBV in the evolution of B-cell clones in T-cell lymphomas.     

Changing incidence of lymphoproliferative disorders in Yorkshire

Secular trends in the incidence of lymphoproliferative disorders on North and West Yorkshire and Humberside from 1985 to 94 were studied and changes in incidence by tumour subtype were analysed. Population-based data on the incidence of lymphoproliferative disorders were obtained from a specialist registry with a high level of ascertainment. Cases of chronic lymphocytic leukaemia and plasma cell myeloma were excluded and the remaining cases classified as Hodgkin's disease and non-Hodgkin's lymphoma (NHL). NHL were subdivided by site of origin and immunophenotype. Nodal B-cell lymphomas were further classified as diffuse large B-cell lymphoma, follicle centre lymphoma, mantle cell lymphoma and miscellaneous. During the study period there was a significant increase in total lymphoproliferative disorders with an average change of 2.5% per annum equivalent to 0.84/10 0000. Most of this increase was due to an increasing incidence of extranodal B-cell lymphomas and peripheral T-cell lymphomas. A numerically small but significant increase in diffuse large B-cell lymphomas was seen. There was no significant increase in other subtypes. The increased incidence of lymphomas in the area studied is mainly due to changes in two specific subgroups. There are several reasons why changes in extranodal B-cell lymphoma and peripheral T-cell lymphoma may have been particularly affected by changing diagnostic practices. Epidemiological studies of particular subtypes of lymphoproliferative disorder facilitate the identification of environmental factors involved in the pathogenesis of these tumours.     

Diffuse large B-cell lymphoma: is morphologic subdivision useful in clinical management?

Abstract: The diffuse large B-cell lymphoma category of the REAL classification encompasses different morphologic lymphoma subtypes in a single entity. The aim of this study is to determine the influence of the morphologic subdivision within this category with respect to clinical features and response to treatment. From January 1993 to October 1996, 132 patients were diagnosed de novo with diffuse large B-cell lymphoma in our institution. All cases were classified according to the REAL and the Updated Kiel classifications, and immunohistochemical study was performed in all of them. Sixty-three per cent of patients received chemotherapy with a curative approach. Of the 105 assessable patients, 80 cases (74%) were classified as centroblastic (CB) and 25 cases (26%) as immunoblastic (IB), according to the updated Kiel classification. These 2 subsets of lymphomas did not differ with respect to major clinical features and laboratory parameters. Both groups had a similar complete response rate with a uniform therapeutic approach and the overall 2-yr survival did not show statistical differences (49% in CB vs. 45% in IB). In conclusion, for clinicians, morphologic subdivision of the diffuse large B-cell lymphoma category into CB and IB subtypes has little clinical and prognostic significance.     

Hepatitis C and risk of lymphoma: results of the European multicenter case-control study EPILYMPH

BACKGROUND & AIMS: Increasing evidence points toward a role of hepatitis C virus (HCV) infection in the etiology of malignant lymphomas. However, previous epidemiologic studies were limited in size to establish an association between HCV infection and specific lymphoma subtypes. We performed a large, multicenter, case-control study to address this question. METHODS: The study comprised 5 European countries and included newly diagnosed cases of any lymphoid malignancy recruited between 1998 and 2004. Controls were matched to cases by 5-year age group, sex, and study center. In-person interviews were conducted to collect data on demographic, medical, and family history as well as environmental exposures. Serum samples of 1807 cases and 1788 controls (excluding human immunodeficiency virus-positive and organ-transplantation subjects) were screened for HCV infection using an enzyme immunoassay. Positive as well as randomly selected negative samples were subjected to HCV RNA detection and HCV genotyping. RESULTS: HCV infection was detected in 53 (2.9%) lymphoma cases and in 41 (2.3%) control subjects (odds ratio [OR], 1.42; 95% confidence interval [CI]: 0.93-2.15). Restricted to individuals who tested positive for HCV-RNA (indicating persistent infection and active viral replication), the OR was 1.82 (95% CI: 1.13-2.91). In subtype-specific analyses, HCV prevalence was associated with diffuse large B-cell lymphoma (OR, 2.19; 95% CI: 1.23-3.91) but not with chronic lymphocytic leukemia or follicular, Hodgkin's, or T-cell lymphoma. The sample size was not sufficient to derive any conclusions for rare lymphoma entities such as splenic marginal zone lymphoma. CONCLUSIONS: These results support a model that chronic HCV replication contributes to lymphomagenesis and establish a specific role of HCV infection in the pathogenesis of diffuse large B-cell lymphoma.