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1.
中线外周T细胞淋巴瘤   总被引:3,自引:0,他引:3  
谢永欣  陈钰  后盾  王爱华  沈杨  沈志祥 《肿瘤》2002,22(6):501-502
目的 总结 13例中线外周T细胞淋巴瘤 (MPTL)探讨其特征、治疗及预后。方法 用病理、免疫酶标方法对 13例MPTL进行确诊 ,采用CHOP方案化疗并观察其预后。结果  8例CR ,2例PR ,其中 2例CR后复发 ,另 3例NR死亡。结论CHOP方案对MPTL有效。血和尿 β2 MG升高和血清LDH升高应视为预后不良因素。  相似文献   

2.
中线外周T细胞淋巴瘤临床分析   总被引:1,自引:0,他引:1  
目的分析中线外周T细胞淋巴瘤临床和病理情况。材料与方法本文回顾性分析自1986~1996年我院经病理证实的中线外周T细胞淋巴瘤39例,原发部位鼻腔27例、扁机体6例、鼻咽4例、软腭2例。其中放化综合治疗25例,单纯放疗8例,化疗5例,未治1例。结果本组资料表明该病以男性为主,病变常累及多个部位,后期可广泛侵犯。B症状多,且易复发、进展,恶性度以中高度为主。单项治疗生存率为40%,综合治疗66.67%,故应做放化综合治疗。如无颈部转移不须做颈部预防放射治疗。本病易短期复发、进展。结论本病恶性度高,作者认为要维持治疗以放化疗综合为主。  相似文献   

3.
中线外周T细胞淋巴瘤19例临床分析   总被引:1,自引:0,他引:1  
目的:分析探讨中线外周T细胞淋巴瘤(midline peripheral T-cell lymphoma,MPTL)的临床特点,诊断,分型,治疗方法的选择和预后。方法:经免疫组化证实的MPTL患者19例。病变累及一个解剖部位的局限型9例。其中8例单纯放射治疗,1例放疗加化疗的综合治疗;病变累及两个及两个以上解剖部位的广泛型10例,其中9例综合治疗,1例单纯化疗化疗方案为CCNU(环己亚硝脲)和CHOP(环磷酰胺。阿霉素,长春新碱,强的松)各5例。结果:19例MPTL患者3年生存率68.4%(13/19)。5年生存率55.6%(5/9)。其中局限型3年生存率88.9%(8/9)。广泛型3年生存率50.0%(5/10)。综合治疗CCNU方案3年生存2/5,CHOP方案3年生存4/5。结论:MPTL是以鼻面中线部位进行性破坏性病变为特征的恶性淋巴瘤。临床表现和病理形态呈现多样化,无特异性。免疫组化染色检查有助本病的诊断。依据病变侵犯的范围可将MPTL分为单一解剖部位受累的局限型和两个及两个以上解剖部位受累的广泛型,前者预后较好。可单纯放射治疗;后者采用放疗加化疗或化疗加放疗的综合治疗可提高远期生存率。辅助化疗以CCNU或CHOP方案为佳。  相似文献   

4.
外周T细胞性淋巴瘤22例病理临床及免疫组化观察   总被引:1,自引:0,他引:1  
许越香  张华忠 《肿瘤》1989,9(2):55-57
T细胞性淋巴瘤在我国约占非何杰金淋巴瘤(NHL)的24.3%~29%。该类肿瘤有多种组织形态及临床表现,至今对T淋巴母细胞性淋巴瘤、蕈样肉芽肿、Sezary氏综合症的病理临床特征已有较全面的认识,而对外周T-NHL的认识尚很不足,在诊断及分类分型上均有不少困难,现将22例外周T-  相似文献   

5.
6.
外周T细胞淋巴瘤的研究进展   总被引:3,自引:1,他引:3  
外周T细胞淋巴瘤(peripheral T-cell lymphomas,PTCL)为异质性血液学肿瘤组,起源于成熟的T细胞,约占全部成人非霍奇金淋巴瘤(NHLs)15%.现今的WHO分类包括9种临床病理学外周T细胞NHLs,其中非特异变异型(PTCL-U)最常见.在诊断时常处于进展期,最常呈侵袭性临床经过,需立即治疗.为更好理解其生物学特性和探索新的治疗方法,本文综述PTCLs(特别着重PTCL-U亚组)的病理学、临床特征和目前的治疗策略.  相似文献   

7.
李小秋 《中国肿瘤临床》2014,41(19):1204-1207
外周T细胞及自然杀伤细胞(T/NK)淋巴瘤是一组少见而又异质、以侵袭性强为特征的恶性肿瘤。由于对该组疾病的病理生物学、特别是遗传和分子生物学改变所知甚少,当前世界卫生组织(WHO)分类根据临床表现将其定义的诸多病种归类到白血病、皮肤肿瘤、结外病变以及淋巴结病变这几组疾病之中。近年来,有关T/NK细胞的亚群构成以及细胞分化方面的研究进展有助于我们更深入了解这些肿瘤的生物学特性。此外,一些新的遗传学改变以及信号路径调节失常也正被发现,这些分子层面的异常,不仅能用作诊断和预后标志物,还是研发治疗新措施的潜在靶点。   相似文献   

8.
高天晓  李志铭 《中国肿瘤临床》2020,47(20):1039-1043
外周T细胞淋巴瘤(peripheral T-cell lymphoma,PTCL)是来源于胸腺后不同阶段的生物学行为及临床表现具有明显异质性的一类恶性淋巴肿瘤。诊断时多为晚期且进展迅速,即使接受高强度的治疗,治愈率仍较低。结外NK/T细胞淋巴瘤(extranodal NK/T-cell lymphoma,ENKTL)为原发于鼻腔或其他结外器官的NK/T细胞淋巴瘤,属于结外非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL)的一种少见特殊类型,占NHL的5%~15%。其发病具有明显的地域和种族差异性,亚洲人群相对高发。ENKTL恶性程度高、临床进展快且预后差。寻求更为标准化、规范化的诊疗指导,学习并理解国际及国内的权威指南尤为重要。本文将对中国临床肿瘤学会(CSCO)淋巴瘤指南以及美国肿瘤学临床实践(NCCN)指南等进行解读,重点对近两年(2019年至2020年)指南中PTCL及自然杀伤T细胞淋巴瘤(natural killer T-cell lymphoma,NKTCL)的部分更改和新增内容进行总结。  相似文献   

9.
外周T细胞与自然杀伤(NK)细胞密切相关,并且具有部分相同的免疫表型和功能特性.外周T和NK细胞淋巴瘤异质性显著,分型复杂,目前大多尚未发现特有的分子表现.但随着分子技术的应用,对血管免疫母细胞T细胞淋巴瘤与辅助T细胞的相关性、间变性大细胞淋巴瘤的基因以及靶向治疗潜在标志物等有了进一步认识.  相似文献   

10.
 外周T细胞淋巴瘤(PTCL)为一组异质性淋巴细胞恶性增殖性疾病,起源于胸腺后成熟T淋巴细胞或自然杀伤细胞。与B细胞淋巴瘤相比,PTCL侵袭性更强,预后更差。综述PTCL的临床诊断与治疗进展。  相似文献   

11.
J X Li 《中华肿瘤杂志》1988,10(5):373-375
Seventy-eight cases of MMR selected from 141 cases of so-called malignant granulomatosis (MG) in our hospital during the past 27 years (1960-1986) are reported. All were treated mainly by radiotherapy, and 50 cases had been followed for long period. The overall 5-year survival rate was 44%. Although most of the patients died within 2 years, two had survived for more than 20 years after treatment. The microscopic features of these lesions were consistent with the peripheral T-cell lymphomas (PTL), which occur in other organs or sites as well. It could be roughly divided into two groups: monomorphic and polymorphic. Monomorphic lesions could be subdivided into several types again, such as large cell, mixed cell, clear cell, intermediate round cell types, and so on. In these cases, 11 were assayed for immunophenotype, including kappa, lambda and ACT, showing that the tumor cells were negative. Furthermore, 3 other new cases were stained with B1, IgM, Kappa, lambda, and/or Leu-2, Leu-3, Leu-4. Two of them were positive for Leu-3+, Leu-4+ and one for Leu-2+, Leu-3+, Leu-4+. All the results conform well to the diagnostic criteria of PTL as agreed upon by some Chinese and foreign authors. That means most of the MMR may be a group of heterogeneous extranodal PTL, and should be treated as T-cell lymphomas.  相似文献   

12.
中线T细胞淋巴瘤与EB病毒关系的研究   总被引:3,自引:0,他引:3  
为了解EB病毒在中线T细胞淋巴瘤中的感染情况,作者对遵义地区46例中线T细胞淋巴瘤进行EBV检测,用聚合酶链反应检测EBV特征性的DNA序列(EBVDNA),用RNA原位杂交法检测EBV编码的RNA(EBER1/2)。结果:EBVDNA阳性率为76.1%(35/46);EBER1/2阳性率为67.4%(31/46)。鼻腔、鼻咽、口咽T淋巴瘤EBER1/2阳性率分别为88.9%(16/18)、71.4%(5/7)、47.6%(10/21)。在多形细胞性淋巴瘤中,中多形和大多形细胞性EBER1/2阳性率为80.0%(16/20),小多形细胞性为73.3%(11/15)。结果表明EBV感染与中线T淋巴瘤关系密切,它在该肿瘤的发病中可能起重要作用,中线T淋巴瘤的EBV感染率与解剖学部位有关,多形细胞性淋巴瘤EBV感染率较高,特别是中多形和大多形细胞性  相似文献   

13.
Pan ZH  Huang HQ  Lin XB  Xia YF  Xia ZJ  Peng YL  Cai QQ  Lin TY  Jiang WQ  Guan ZZ 《癌症》2005,24(12):1493-1497
背景与目的:鼻型NK/T细胞淋巴瘤(NK/T-celllymphoma,NK/TCL)是一种特殊类型的淋巴瘤,常有局部坏死穿孔、感染和发热,单用化疗效果不佳,以放化疗综合治疗效果较好。本研究目的是分析鼻型NK/T细胞淋巴瘤的临床特征、近期疗效、远期生存以及影响预后的因素,为诊断和治疗提供新经验。方法:回顾性分析中山大学肿瘤防治中心1997年1月至2004年6月间收治的93例鼻型NK/T细胞淋巴瘤的临床特征、疗效和不良反应。采用Kaplan-Meier法进行生存分析,COX模型进行多因素分析。结果:93例患者中Ⅰ Ⅱ期75例(80.6%),Ⅲ Ⅳ期18例(19.4%);就诊时主要症状为鼻塞、流涕和鼻出血等,确诊前病程1~24个月,中位病程6.5个月;临床检查发现有硬腭和/或鼻中隔穿孔表现者15例(16.1%),35例(37.6%)有发热等B症状。37例(39.8%)采用单一治疗(单纯化疗35例、单纯放疗2例),54例(58.0%)采用化、放疗联合,2例(2.2%)未行任何治疗;一线化疗多采用CHOP类方案或EPOCH方案。全组总有效率84.4%(76/90),CR率64.4%(58/90);单纯化疗有效率67.6%(23/34),CR率41.2%(14/34);放疗加化疗有效率94.4%(51/54),CR率83.3%(45/54),2例未行任何治疗者均在半年内死亡。化疗主要不良反应为骨髓抑制,Ⅲ~Ⅳ度粒细胞减少、血小板减少和贫血的发生率分别为37.7%、13.7%和10.7%;放疗不良反应主要为Ⅰ~Ⅱ度粘膜损伤和骨髓抑制;其他不良反应少见。全组死亡62例(66.7%),1、3和5年生存率分别为63.4%、43.1%和17.6%。多因素分析结果显示,硬腭和/或鼻中隔穿孔、B症状和治疗模式为独立的预后因素(P值分别为0.035、0.000和0.004)。结论:对鼻型NK/T淋巴瘤采用单纯化疗疗效较差,而化、放疗联合疗效较好,但远期生存情况仍不满意,需寻找提高鼻型NK/T细胞淋巴瘤疗效的治疗方案。  相似文献   

14.
PURPOSE: To describe and identify the clinical and pathologic features of prognostic significance for natural killer (NK) and NK-like T-cell (NK/T-cell) lymphoma presenting in the skin. PATIENTS AND METHODS: This study was a retrospective review of 30 patients with CD56+ lymphomas initially presenting with cutaneous lesions, with analysis of clinical and histopathologic parameters. RESULTS: The median survival for all patients was 15 months. Those with extracutaneous manifestations at presentation (11 patients) had a shorter median survival of 7.6 months as compared with those without extracutaneous involvement (17 patients), who had a more favorable median survival of 44.9 months (P =.0001). Age, gender, extent of cutaneous involvement, and initial response to therapy had no statistically significant effect on survival. Seven patients (24%) had detectable Epstein-Barr virus (EBV) within neoplastic cells. The patients with tumor cells that coexpress CD30 (seven patients) have not yet reached a median survival after 35 months of follow-up as compared with those with CD30- tumor cells (20 patients), who had a median survival of 9.6 months (P <.02). Routine histopathologic characteristics had no prognostic significance nor did the presence of CD3epsilon, EBV, or multidrug resistance. CONCLUSION: NK/T-cell lymphoma is an aggressive neoplasm; however, a subset with a more favorable outcome is identified in this study. The presence of extracutaneous disease at presentation is the most important clinical variable and portends a poor prognosis. The extent of initial skin involvement does not reliably predict outcome. Patients from the United States with NK/T-cell lymphoma presenting in the skin have a low incidence of demonstrable EBV in their tumor cells. Patients with coexpression of CD30 in CD56 lymphomas tend to have a more favorable outcome.  相似文献   

15.
The humanized monoclonal antibody CAMPATH-1H (alemtuzumab) binds to the CD52 antigen, a glycoprotein that is widely expressed on normal and malignant B- and T-lymphocytes. Over the past 5 years, a number of trials have demonstrated that alemtuzumab has clinical activity in mature T-cell diseases such as T-cell prolymphocytic leukemia (T-PLL) and cutaneous T-cell lymphoma (CTCL). In heavily pretreated relapsed/refractory patients alemtuzumab induced responses in more than two thirds of T-PLL and more than 50% of CTCL patients. Responding patients had improved survival compared to nonresponders. Alemtuzumab is particularly effective in clearing malignant lymphocytes from peripheral blood and bone marrow and may therefore facilitate stem-cell transplantation (SCT) in selected patients. The toxicity profile for the antibody is acceptable; the major complications are infusional reactions, which generally subside after the first 1-2 weeks of therapy, and prolonged lymphopenia associated with reactivation of viruses. These can be minimized by careful monitoring and the use of prophylactic therapy. Future studies will be directed toward: alternative routes (subcutaneous) and schedules of administration; use as first-line therapy; combination strategies with conventional chemotherapy; and use of alemtuzumab to purge minimal residual bone-marrow disease prior to SCT.  相似文献   

16.
Endobronchial involvement by non-Hodgkin's lymphoma (NHL) is uncommon and usually occurs in the presence of more generalised disease. Solitary endobronchial lymphoma in the absence of disease elsewhere is extremely rare. In this report we describe a patient with an obstructing endobronchial mass which was the initial manifestation of NHL. The patient was treated initially with radiotherapy followed by cyclic combination chemotherapy with initial complete resolution of the endobronchial disease. However, he subsequently developed widespread lymphoma. This unusual presentation of NHL is discussed and the relevant literature is reviewed.  相似文献   

17.
PURPOSE OF REVIEW: Peripheral T-cell lymphomas are an uncommon and heterogeneous group of non-Hodgkin's lymphomas that are noted for their particularly poor prognosis. Their rarity has resulted in few data being available to allow formulation of optimal treatment approaches. There remains no widely accepted standard therapy. A new and increasing interest in studying these lymphomas is leading to advances in our understanding, which is widening options for management. RECENT FINDINGS: Historically, peripheral T-cell lymphomas were treated with strategies borrowed from management of aggressive B-cell lymphomas. Investigators have recognized the inadequacy of these approaches, and we are beginning to receive results, often preliminary, of studies specifically designed to evaluate T-cell lymphomas. These range from transplantation approaches, to better prognostic schemes and to attempts at molecular characterization, and new drugs are being developed specifically for their activity against T-cell lymphomas. SUMMARY: Research in T-cell lymphomas is still mainly preliminary and studies tend to be small. Nonetheless, our understanding of these disorders is increasing, and data on new and hopefully better approaches are emerging.  相似文献   

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