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1.
血管免疫母细胞性T细胞淋巴瘤(T-AILT)是较罕见的外周T细胞肿瘤,占非霍奇金淋巴瘤(NHL)1%~2%,占外周T细胞淋巴瘤15%~20%[1].AILT具有特殊的临床表现、形态学特征和免疫表型,过去认为是一种非恶性免疫性疾病,称为血管免疫母细胞性淋巴结病(AILD).近年来研究证实,AILT是一种独立的侵袭性外周T细胞肿瘤.故WHO(2001)淋巴造血组织肿瘤分类中已将AILD归为AILT.本文分析我院收治的1例AILT,探讨该病的临床病理特征、诊断及治疗.  相似文献   

2.
恶性淋巴瘤是来源于淋巴造血系统的恶性肿瘤,其发病率近年增长迅速,已位居我国常见恶性肿瘤的前10位。随着对淋巴瘤研究的不断深入,目前在其病理诊断与分型、分期和治疗方法等方面均有了较大进展。在目前广泛使用的 WHO 分类中[1],恶性淋巴瘤分为霍奇金淋巴瘤(HL)和非霍奇金淋巴瘤(NHL)两大类。HL 分为结节性淋巴细胞为主型和经典型,经典型包括结节硬化型、富于淋巴细胞型、混合细胞型及淋巴细胞消减型4种亚型,中国人以混合细胞型和结节硬化型最为多见。HL 在我国发病率<10%,形态相对典型,标记也比较特异,一直被大家认为诊断准确率很高,但是也不尽然。NHL 分为 T 细胞和 B 细胞淋巴瘤两类,每一类根据肿瘤细胞成熟程度又分为前驱淋巴细胞肿瘤和成熟淋巴细胞肿瘤,前驱淋巴细胞肿瘤多见于儿童和青少年,老年人中也可见。T 或 B 细胞淋巴瘤根据不同的组织形态学、免疫表型及分子遗传学特点各分为几十种亚类。中国常见的 T 细胞淋巴瘤为 NK/T 细胞淋巴瘤(NK/TCL)、间变性大细胞淋巴瘤(ALCL)、血管免疫母细胞性 T 细胞淋巴瘤(AITCL)、外周 T 细胞淋巴瘤(PTCL)及淋巴母细胞淋巴瘤/白血病(LBL/ALL)等。常见的 B细胞淋巴瘤为弥漫性大 B 细胞淋巴瘤(DLBCL)、结外边缘区 B 细胞淋巴瘤、黏膜相关淋巴组织细胞型(MZL-MALToma)及滤泡性淋巴瘤(FL)[2]。不同类型或亚型淋巴瘤的临床表现、治疗和预后各不相同,即使同一类型淋巴瘤的不同形态学变异,其生物学行为也会有差异,从而导致临床治疗选择的不同。因此,恶性淋巴瘤的诊断和正确分类具有重要的临床意义。  相似文献   

3.
目的 探讨肺原发性支气管相关淋巴组织(BALT)淋巴瘤的临床诊断及治疗,提高对BALT淋巴瘤的认识.方法 分析肺原发性BALT淋巴瘤的临床特点、影像学表现、病理特征、临床诊断,并复习文献.结果 患者以反复咳嗽、咳痰伴胸闷、气促为其特点;胸部CT检查显示:双肺多发大片实变影,右侧胸腔积液;CT引导下经皮肺穿刺活检病理检查结果:黏膜相关淋巴组织结外边缘带小B淋巴细胞淋巴瘤,免疫表型:CD20、BCL-2、ki-67、PAX-5均阳性;CD3、CD5、CD10、CD23、CD43、cyclin-D1、MPO等表达均阴性.结论 BALT淋巴瘤临床罕见,属于低度恶性B细胞淋巴瘤,依靠组织细胞学病理、免疫表型与临床、影像学相结合可明确诊断.  相似文献   

4.
目的回顾性总结分析肺淋巴瘤的分类、分型特征及临床特点。方法收集北京协和医院1999年-2016年近18年期间病理诊断为肺淋巴瘤的全部病例,通过光镜、免疫组织化学染色及基因重排方法进行病理分析。结果本组共有肺淋巴瘤病例142例,男女比例1︰1.33,平均年龄48岁。123例(86.6%)诊断为非霍奇金淋巴瘤,19例(13.4%)诊断为霍奇金淋巴瘤。101例(69.2%)诊断为B细胞性淋巴瘤,其中45.6%(46例)为黏膜相关淋巴组织淋巴瘤或边缘区淋巴瘤,18.8%(19例)为弥漫大B细胞性淋巴瘤,11.9%(12例)为淋巴瘤样肉芽肿病。22例诊断为T细胞性淋巴瘤,其中27.3%为外周T细胞淋巴瘤(6例),13.6%(3例)为NK/T细胞淋巴瘤,9.1%(2例)为间变大细胞淋巴瘤。121例患者临床资料完善,其中64例(52.9%)为原发性肺淋巴瘤,男女比例1︰1.37,平均年龄50岁;57例(47.1%)为继发性肺淋巴瘤,男女比例1︰1.19,平均年龄45岁。在原发性肺淋巴瘤中,96.9%为非霍奇金淋巴瘤,3.1%为霍奇金淋巴瘤;在继发性肺淋巴瘤中,78.9%为非霍奇金淋巴瘤,21.1%为霍奇金淋巴瘤。65例手术切除或胸腔镜下肺活检病例全部进行了病理分类及分型,77例穿刺活检病例中有22例(28.6%)进一步分型困难。结论肺淋巴瘤中最常见类型依次为黏膜相关淋巴组织淋巴瘤、霍奇金淋巴瘤、弥漫大B细胞性淋巴瘤和淋巴瘤样肉芽肿病,其中黏膜相关淋巴组织淋巴瘤和淋巴瘤样肉芽肿病是主要的原发性肺淋巴瘤类型,而弥漫大B细胞性淋巴瘤和霍奇金淋巴瘤是主要的继发性肺淋巴瘤类型。开胸手术或胸腔镜下肺活检的病例易于明确病理分型,而穿刺活检病例中有小部分病例进一步分型困难。  相似文献   

5.
外周T细胞淋巴瘤临床分析   总被引:1,自引:0,他引:1  
2001年颁布的WHO淋巴肿瘤新分类中认为外周T细胞淋巴瘤-非特指型(PTCL-U)是来源于胸腺后T淋巴细胞的一大类恶性肿瘤。这是一类排除性疾病,它包括除特指的外周T细胞以外的所有不能分型的外周T细胞淋巴瘤,即诊断前需除外T/NK细胞淋巴瘤、淋巴母细胞淋巴瘤、间变大细胞淋巴瘤等。本病有明显的区域分布,在欧美国家发病率较低,约占非霍奇金淋巴瘤(NHL)7%,而在我国及亚洲国家发病率较高,约占T细胞NHL的50%,占NHL的15%。目前,对PTCL-U的临床表现、病理形态、免疫学及细胞遗传学的研究有限,认识不足,近年来受到国内外学者的关注。现将我院2001年7月至2004年6月的23例PTCL—U进行回顾性分析,探讨其临床表现、诊断、治疗及预后。  相似文献   

6.
胃肠道惰性T细胞淋巴组织增殖性疾病(ITLPD-GI)是一种低度恶性的克隆性T细胞增生性疾病, 以惰性行为和慢性复发性为临床基本特征, 较为罕见, 世界卫生组织将其列为肠道T细胞淋巴瘤亚型之一。ITLPD-GI具有独特的临床病理特征, 但由于其罕见性且临床医师对其形态学特征认识不足, 易被误诊为炎症性肠病或具有侵袭性的T细胞淋巴瘤等。现对ITLPD-GI的临床特征、形态学表现、遗传学改变、免疫表型和临床诊治进行综述, 以期提高临床医师对ITLPD-GI的认识。  相似文献   

7.
小 B 细胞淋巴瘤是一组与弥漫大 B 细胞淋巴瘤(DLBCL)相对的、主要由中、小 B 淋巴细胞构成的肿瘤占非霍奇金淋巴瘤(NHL)的46.3%,B 细胞淋巴瘤(BCL)的56.7%,包括滤泡淋巴瘤(FL)、套细胞淋巴瘤(MCL)、小淋巴细胞淋巴瘤(SLL)、淋巴结边缘区 B细胞淋巴瘤(NMZL)、结外黏膜相关淋巴组织边缘区B 细胞淋巴瘤(MALToma)、脾边缘区淋巴瘤(SMZL)和淋巴浆细胞淋巴瘤(LPL)等。2008年 WHO 恶性淋巴瘤分类将这几种淋巴瘤都归于各自具有临床表现、形态学、免疫表型、遗传学特点的独立疾病[1]。临床实践中各种不同类型小 B 细胞淋巴瘤的诊断常会遇到困难,而诊断正确与否直接关系到治疗时机、治疗方案选择和预后评估。我们对各种小 B 细胞淋巴瘤的临床表现、细胞形态、免疫表型、遗传学特征进行总结。  相似文献   

8.
目的:探讨原发性肝黏膜相关淋巴组织(MALT)淋巴瘤的临床病理特征及治疗方法。方法:对1例原发性肝MALT淋巴瘤的发病因素、临床表现、病理特征及治疗结果进行分析。结果:腹部B超检查发现肝左叶低回声区。腹部MRI检查示肝脏恶性占位性病变,行肝左叶切除术,病理检查示正常肝小叶结构破坏,被弥漫分布的肿瘤细胞替代,瘤细胞以单核样细胞为主,伴有淋巴上皮病变形成,免疫组化示,CD45、CD20、CD79a阳性,CD5、CD10、ALK、TdT阴性,HCV、HBV和EBV阴性。结论:原发性肝MALT淋巴瘤属低度恶性B细胞淋巴瘤,有独特的临床、组织病理学和免疫表型特点。治疗上多采取手术局部切除治疗,部分需加用联合化疗。  相似文献   

9.
张纯  陈燕 《临床内科杂志》2007,24(4):271-272
目的探讨肝脾γδT细胞淋巴瘤的临床病理及免疫分型特征。方法应用常规病理、免疫组化及PCR方法观察1例肝脾γδT细胞淋巴瘤,并进行相关文献复习。结果患者以肝大、巨脾、发热为主要表现,肝脾活检病理结果显示淋巴瘤细胞侵犯脾红髓、肝窦及汇管区;免疫组化显示瘤细胞表达CD3、CD45RO、CD15,而CD20、CD79α表达阴性;PCR方法发现TCRγ链重排;且患者存在溶血及血小板减少,诊断符合肝脾γδT细胞淋巴瘤合并Evans综合征。结论肝脾γδT细胞淋巴瘤是罕见的淋巴瘤类型,具有独特的临床病理特征和免疫表型,应注意与其他造血系统疾病鉴别。Evans综合征是其少见的并发症之一,可能与自身免疫异常有关。  相似文献   

10.
目的探讨眼结膜黏膜相关淋巴组织边缘区淋巴瘤临床病理特征、诊断、治疗及预后。方法回顾性分析了11例原发性眼结膜黏膜相关淋巴组织边缘区淋巴瘤患者的临床、病理资料并进行了随访。结果11例患者中男4例,女7例,发病年龄23~74岁,平均年龄为45岁。累及单侧结膜10例,双侧结膜1例,其他部位及系统未见淋巴瘤。病理形态:结膜固有层内弥漫性小-中等大小的淋巴样细胞及单核样细胞浸润,核形轻度不规则。免疫表型:弥漫性浸润细胞CD20(+)、CD3(-)、CD5(-)、CD10(-)、CD23(-)、Cyclin D1(-),5例异常表达CD43,ki67增殖指数为1%~10%。5例行Ig基因克隆性分析,均呈单克隆。6例单纯肿物完整切除,2例肿物完整切除加局部放疗,1例肿物部分切除加局部放疗,1例单纯活检,1例活检后局部注射化疗,肿物未消退,随后行肿物完整切除加局部放疗。随访时间3~122个月,截止随访日期,除2例失访,其他患者生存。其中行肿物单纯活检的患者长期带瘤生存,余8例未见复发。结论眼结膜黏膜相关淋巴组织边缘区淋巴瘤好发于中年女性,结膜见粉红色肿物为临床特征,镜下为小-中等大小的淋巴样细胞及单核样细胞弥漫性浸润,肿瘤细胞表达B淋巴细胞表型,增殖指数低,部分病例异常表达CD43,Ig基因克隆性分析有助于诊断,单纯肿物完整切除或术后辅以局部放疗均有较好的疗效,预后良好。  相似文献   

11.
BACKGROUND: The purpose of the present paper was to investigate the clinical, endoscopic and histological features of 31 patients with gastric mucosa-associated lymphoid tissue (MALT) lymphoma to enable correct, early stage diagnosis. METHODS: A retrospective study was undertaken of 31 patients with gastric MALT lymphoma. The cases were examined immunohistologically with anti-CD(20CY) and CD(45RO) antibodies for further diagnosis. Helicobacter pylori infection was also detected with modified Giemsa staining. RESULTS: Patients with MALT lymphoma were aged between 22 and 73 years (mean, 45.0 years), and the male:female ratio was 11:20. The patients presented with non-specific symptoms, but chronic epigastric pain was the common symptom in a large proportion of the cases. The gastric smaller curvature was involved in 83.9% of cases (26/31) and in 13/31 cases (41.9%) it was confined the antrum. Under endoscopy, large and deep ulcers were similar to cancers in the majority of patients. Only 29.0% of patients were diagnosed by endoscopy on first examination. CD(20CY) were expressed in all cases and CD(45RO) expressed in only one case among 10 cases of indefinite diagnosis. Helicobacter pylori infection was found in 87.1% of patients. CONCLUSIONS: These findings suggest that primary gastric MALT lymphoma has unique clinical, endoscopic and histological features. The diagnosis for primary gastric MALT lymphoma was delayed not only due to the non-specific symptoms but also due to lack of attention to its features. Endoscopy and submucosal multiple biopsy were the principal diagnostic tools in patients with gastric MALT lymphoma. CD(20CY) and CD(45RO) immunological staining are recommended, especially for patients with indefinite diagnosis of gastric MALT lymphoma.  相似文献   

12.
Mucosa‐associated lymphoid tissue (MALT) lymphoma is the most frequent non‐Hodgkin lymphoma in the gastrointestinal tract, but colon involvement has only been reported in multiorgan lymphoma. We present a rare case of a woman with MALT involvement of eye conjunctiva, tonsils, stomach, duodenum and colon. In selected cases like this, with multiorgan involvement, we recommend performing colonoscopy, with biopsies for immunohistochemistry with CD10 and cyclin D1 for differential diagnosis with other entities with different prognosis, such as follicular lymphoma, and mantle cell lymphoma, respectively.  相似文献   

13.
We report a case of composite lymphoma consisting of peripheral T-cell lymphoma and an anaplastic variant of diffuse large B-cell lymphoma (DLBCL) and associated with Epstein-Barr virus (EBV) infection and strong p53 expression. A 65-year-old Japanese woman developed fever and generalized lymphadenopathy. A biopsy of the cervical node revealed the morphology of malignant lymphoma with 2 kinds of lymphoma coexisting in 1 lymph node. One lymphoma type consisted of immunoblastic large cells with the T-cell marker phenotype CD3+, CD45RO/UCHL-1+, CD20/L26-, CD79-, CD10-, CD30-, and CD15-; the other type consisted of large cells with abundant cytoplasm and pleomorphic nuclei with the marker phenotype CD79+, CD20/L26+, CD45RO/UCHL-1-, CD3-, CD10-, CD30+, NPM/ALK-, and CD15-. Therefore, the diagnosis was composite lymphoma of peripheral T-cell lymphoma and an anaplastic variant of DLBCL, stage IVB, because the patient had bone marrow involvement with peripheral T-cell lymphoma. The biopsy led to findings of latent type II EBV-associated lymphoma in both the peripheral T-cell lymphoma and the anaplastic variant of DLBCL as the result of positive signals for EBV small RNAs by in situ hybridization, positive immunostaining results for EBV latent membrane protein 1 antibody, and negative immunostaining results for EBV nuclear antigen 2. Immunostaining of the mass with p53 antibody also yielded positive results for both types of lymphoma cells. This case suggests that the immunocompromised state of this patient with EBV-related peripheral T-cell lymphoma allowed the emergence of an EBV-related anaplastic variant of DLBCL and suggests a close relationship between p53 expression and latent EBV infection.  相似文献   

14.
15.
BACKGROUND: Little is known about the function of T cells in the inflammatory infiltrate in Helicobacter pylori-associated gastritis and B-cell lymphoma of mucosa-associated lymphoid tissue (MALT type). Previous studies have proposed a dominant Th1-type response in low-grade MALT lymphoma consistent with the Th1 response observed in H. pylori-associated gastritis. METHODS: We performed a novel flow cytometric approach in which CD3 panning for enrichment and activation of small numbers of T cells and intracellular cytokine analysis were combined to selectively characterize the cytokine profile of T cells (IFN-gamma for Th1) derived from the gastric mucosa of 23 patients with low-grade MALT lymphoma stage IEI1 (lymphoma infiltration of mucosa/submucosa sparing the muscularis). Endosonography was performed in each case to control the depth of lymphoma infiltration. For comparison, 19 patients with H. pylori-positive gastritis were also analysed. RESULTS: There was a CD4/CD8 ratio of 4 in patients with MALT lymphoma and of 2 in chronic gastritis. The proportion of IFN-gamma producing cells within the CD4-positive T-cell population in MALT lymphoma was 22%; in chronic gastritis it was 13% while no such difference could be encountered in CD8-positive T cells. CONCLUSIONS: The data point towards a dominant intratumoral IFN-gamma dominated T-cell response associated with early low-grade MALT lymphoma. A polarized IFN-gamma dominated Th1-type response may either contribute to the inability of the immune system to eradicate H. pylori infection, thereby promoting the activation status of the lymphocytic infiltrate in low-grade MALT lymphoma, or may mirror a concomitant tumor-specific T-cell response accompanying early stages of tumor progression.  相似文献   

16.
Primary hepatic lymphoma, mostly diffuse large B-cell lymphoma, is a rare disease. We describe an extremely rare case of low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type occurring in the liver. A 61-year-old man with a history of hepatitis A presented with early gastric cancer and a liver mass. Needle biopsy of the liver tumor suggested low-grade B-cell lymphoma by histology and polymerase chain reaction of the immunoglobulin heavy chain gene. The tumor (3.4 x 2.8 x 2.4 cm) was completely resected from the anterior segment of the right lobe of the liver. Atypical lymphoid cells of small to intermediate size proliferated in the tumor, and lymphoepithelial lesions were recognized. Immunohistochemically, lymphoma cells were positive for CD20 and negative for CD5, CD10, and cyclin D1. Staging procedures showed no lymphoma lesion other than the liver tumor. Thus, the patient was diagnosed with low-grade hepatic marginal zone B-cell lymphoma of the MALT type. The patient has been followed up for 1.5 years since surgical resection with no recurrence. The clinicopathologic characteristics and management of this rare disease are discussed.  相似文献   

17.
Background: Little is known about the function of T cells in the inflammatory infiltrate in Helicobacter pylori -associated gastritis and B-cell lymphoma of mucosa-associated lymphoid tissue (MALT type). Previous studies have proposed a dominant Th1-type response in low-grade MALT lymphoma consistent with the Th1 response observed in H. pylori -associated gastritis. Methods: We performed a novel flow cytometric approach in which CD3 panning for enrichment and activation of small numbers of T cells and intracellular cytokine analysis were combined to selectively characterize the cytokine profile of T cells (IFN- &#110 for Th1) derived from the gastric mucosa of 23 patients with low-grade MALT lymphoma stage IEI 1 (lymphoma infiltration of mucosa/submucosa sparing the muscularis). Endosonography was performed in each case to control the depth of lymphoma infiltration. For comparison, 19 patients with H. pylori -positive gastritis were also analysed. Results: There was a CD4/CD8 ratio of 4 in patients with MALT lymphoma and of 2 in chronic gastritis. The proportion of IFN- &#110 producing cells within the CD4- positive T-cell population in MALT lymphoma was 22%; in chronic gastritis it was 13% while no such difference could be encountered in CD8-positive T cells. Conclusions: The data point towards a dominant intratumoral IFN- &#110 dominated T-cell response associated with early low-grade MALT lymphoma. A polarized IFN- &#110 dominated Th1-type response may either contribute to the inability of the immune system to eradicate H. pylori infection, thereby promoting the activation status of the lymphocytic infiltrate in low-grade MALT lymphoma, or may mirror a concomitant tumor-specific T-cell response accompanying early stages of tumor progression.  相似文献   

18.
 Mucosa-associated lymphoid tissue (MALT) lymphomas are indolent neoplasms which tend to remain localized for a long time before spreading. We describe here the case of a 36-year-old woman with a low-grade MALT lymphoma involving the lung, stomach, lingual tonsil, and bone marrow at the time of diagnosis. The clonal origin of the pulmonary and bone marrow neoplastic infiltrates was assessed by means of gene rearrangement analysis. All of the involved sites were infiltrated by centrocyte- and monocytoid-like cells expressing the B-cell-associated antigens CD19 and CD20 and showed IgM λ chain restriction; no CD5, CD10, or CD43 expression was detectable. As the patient had a history of recurrent bronchitis, and computed tomography performed 3 years before the lymphoma diagnosis had already revealed a lesion of the left lung, we conclude that the present case probably represents a pulmonary low-grade MALT lymphoma characterized by an early and unusual involvement of different mucosal sites and bone marrow. Received: 27 June 1997 / Accepted: 20 November 1997  相似文献   

19.
A patient presented with lymphoblastic lymphoma in lymph-nodes and chronic myeloge-nous leukemia (CML) in marrow and peripheral blood. All marrow and unstimulated peripheral blood cells contained the Philadelphia chromosome{t(9:22)}. Lymphoma cells were analyzed by flow cytometry and were identified as T cells (CD2+CD5+CD7+CD34+). All fresh lymphoma cells contained the t(9:22) translocation. Cultures of purified peripheral blood T and B cells and specifically stimulated NK cells revealed that 59% of the B cells, 10% of the NK cells, and none of the normal T cells contained the translocation. The lack of translocation in normal peripheral T cells is attributed to their long lifespan. No rearrangement of immunoglobulin or T cell receptor beta or gamma genes was found in either the leukemia or lymphoma cells. Analysis of the DNA from cryopreserved lymphoma biopsy showed clonal rearrangement within the common breakpoint cluster region of the bcr gene identical to the bcr rearrangement in DNA from leukemia blood cells. The data support the concept that T and B cells originate in the patient's totipotent stem cell from which the CML is also derived. © 1993 Wiley-Liss, Inc.  相似文献   

20.
Introduction: Peripheral T‐cell lymphomas (PTCL) represent approximately 10% of non‐Hodgkin's lymphomas. Pulmonary involvement is an uncommon manifestation of this heterogeneous group of malignancies. Methods: Report of a case. Results: This case report describes a 75‐year‐old man with fever, weight loss, anemia, enlargement of spleen and liver, atypical lymphocytes and pulmonary nodules. Lung biopsy showed lymphocytic infiltration of the lung parenchyma. T‐cell receptor gamma gene rearrangement by polymerase chain reaction confirmed the diagnosis of peripheral T‐cell lymphoma. Unfortunately, the patient died because of refractory and aggressive disease. Conclusion: Pulmonary and pleural involvement are seen in patients with PTCL and usually carry a poor prognosis. The subject of pulmonary involvement in peripheral T‐cell lymphoma is discussed. Please cite this paper as: Vahid B, Machare‐Delgado E and Marik PB. Pulmonary manifestations of peripheral T‐cell lymphoma: case report and review of the literature. The Clinical Respiratory Journal 2007; 1:114–117.  相似文献   

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