肠病相关T细胞淋巴瘤7例临床特征分析

目的探讨肠病相关T细胞淋巴瘤(EATL)的临床特点及其治疗策略。方法回顾性分析南京医科大学第一附属医院血液科7例初诊EATL患者的临床资料,探讨其治疗方案。结果 7例既往均无肠道疾病病史,均因消化道症状就诊。Ann Arbor及Lugano分期均以Ⅰ+Ⅱ期为主(6/7,85.7%)。6例完成6个疗程化疗,最大疗效均达完全缓解(CR),其中1例予自体造血干细胞移植(ASCT)巩固治疗。中位随访时间13个月(1~25个月),5例(包括ASCT)死亡。2年总生存率为25.7%。结论 EATL呈高度侵袭性,化疗可使患者短期获益,但容易复发。采用ASCT作为一线巩固治疗也难以获得长期疗效,异基因造血干细胞移植作为一线巩固治疗值得进一步探讨。     

肠病型T细胞淋巴瘤内镜典型形态--附6例临床分析

肠病型T细胞淋巴瘤(Enteropathy-type intestinal T cell lymphoma,ETCL)亦称肠病相关性淋巴瘤,是原发性肠道的结外型恶性淋巴瘤,恶性程度极高,本病少见,以前曾误称为肠型恶性组织细胞增生症(肠型恶组),近年来病理界已明确其来源于NK/T细胞,属外周T细胞型恶性淋巴瘤.     

肠病相关的T细胞性淋巴瘤

随着免疫学的发展，人们对肠道原发淋巴瘤有了新的认识，提出了肠病相关T细胞性淋巴瘤（enteropathy－as－sociatedTcelllymphoma，EATCL）的新概念‘－”。认为传统观念中的肠道恶性组织细胞增生症（恶组）实际上是EATCL。本研究应用多种单克隆抗体，在石蜡切片上对14例既往被诊断为肠道恶组，而经免疫组化证实为T细胞来源肿瘤的病例进行研究，探讨其组织起源，并对其临床表现和病理形态学特点进行了分析。材料和方法14例病理标本均为南方医院病理科存档材料，外科手术标本8例，尸检标本6例。对临床资料进行归纳总结，选取典型病变蜡…     

原发性肠道T细胞淋巴瘤误诊为炎症性肠病的原因分析

目的原发性肠道T细胞淋巴瘤和炎症性肠病临床表现和影像学表现相似，为了预防误诊误治，我们对被误诊为炎症性肠病的原发性肠道T细胞淋巴瘤的临床特征进行分析。方法收集我院十年来（1994—2004年）所有肠道T细胞淋巴瘤和曾被误诊为炎症性肠病的原发性肠道T细胞淋巴瘤的资料，共6例进行分析。结果肠道T细胞淋巴瘤临床上少见，原发性肠道T细胞淋巴瘤和炎症性肠病在临床表现影像学和内窥镜的大体表现颇为相似，内窥镜下的活检和病理学和免疫组化检查是目前确诊肠道T细胞淋巴瘤的主要依据。结论肠道T细胞淋巴瘤的发病率低使临床医生对此种疾病的认识不足。由于缺乏特异性检测手段，为了避免误诊炎症性肠病，提高肠道T细胞淋巴瘤的诊断，要注意以发热、腹泻、腹痛、腹胀等非特异性症状为主，治疗效果不佳的患者要考虑原发性肠道T细胞淋巴瘤的可能性。内窥镜下大多表现为多形性、多灶性、弥漫性和不规则性溃疡，要在有特征性表现的部位多取组织块，提高活检的阳性率。肠道T细胞淋巴瘤易并发消化道穿孔、肠梗阻、消化道出血，诊断不明确时，必要时尽早剖腹探查以明确诊断。     

肺部症状首发的肠病型T细胞淋巴瘤2例并文献复习

目的 提高对以肺部症状为首发的肠病型T细胞淋巴瘤的认识.方法 收集2例以肺部症状首发的肠病型T细胞淋巴瘤,并结合相关文献对其临床表现、诊断思路及治疗方法进行分析总结.结果 仅以肺部症状首发的肠病型T细胞淋巴瘤较为少见,不易诊断.临床表现主要为反复无规律性发热、咳嗽,后期可伴腹部症状(肠出血、肠穿孔).纤维支气管镜和经皮...     

肠病型T细胞淋巴瘤一例及文献复习

一、病历资料 患者男，54岁。因胸闷，气短1个月，腹胀20d，双下肢水肿10d入院。1个月前无诱因出现胸闷、气急，活动后明显，无胸痛发热，无咳嗽咯痰，夜间可平卧。当地医院查B超示心包腔内有液性暗区、肝肿大；胸片示心影扩大；血常规正常；心电图示V1～V5导联T波低平；诊断为“结核性心包炎”，予抗结核、氟美松治疗20d，胸闷气急减轻，但仍有腹胀、双下肢水肿转我院。     

肠病型T细胞淋巴瘤临床分析(附5例报道)

肠病型T细胞淋巴瘤(ETCL)是一种临床少见、预后差的疾病,了解其临床病理特征对诊断和治疗有重要的临床意义.目的:通过分析ETCL患者的临床表现、病理改变和分子生物学特征及其与EB病毒(EBV)感染的关系,提高临床医师和病理医师对ETCL的认识,以期改善患者的生存率.方法:回顾我院2001年7月～2004年1收治的5例ETCL患者的内镜活检或手术切除标本的临床和组织病理学表现;采用免疫组化方法标记CD45RO、CD3、CD56、CD20、CD79α、CD68、Lys;采用聚合酶链反应(PCR)检测EBV感染和T细胞受体(TCR)-γ基因重排情况.结果:5例ETCL患者均无肠病史,多发生于青年男性,4例发病年龄为24～38岁,病程进展迅猛,确诊后4个月内死亡.病变多发生于结肠和小肠,1例与胃部同时发生,均为多发性溃疡性病变.肿瘤细胞为多形性T细胞,组织学形态为多形性淋巴细胞弥散分布,有血管中心性浸润,淋巴上皮病变,大片或多灶性坏死为特征.5例患者肿瘤组织CD45RO和TCR-γ基因重排均为阳性,1例CD56阳性且EBV阳性.结论:ETCL多发生于青年男性,临床症状多无特异性,病程发展迅猛,预后差,病损肠管表现为多发性溃疡,TCR-γ链克隆性重排,多数为自然杀伤(NK)样T细胞淋巴瘤和外周T细胞淋巴瘤非特殊型;有EBV感染的ETCL为NK样T细胞淋巴瘤.     

以直肠溃疡和低蛋白血症为主要表现的肠病相关T细胞淋巴瘤

肠病相关T细胞淋巴瘤(EATL)发病率低,其临床和影像学表现均无明显特异性,需依靠病理学检查才能被确诊,在临床工作中极易被漏诊或误诊。本例患者以直肠溃疡性质待定被收治入院,经多学科协作会诊,最终确诊为首发部位在直肠的EATL。现分享本病例的诊治思路,为临床EATL病例的诊治提供参考。     

小肠结外NK/T细胞淋巴瘤1例报告

患者男性,46岁,因发热40 d间断腹痛15 d入院。患者于40 d前无明显诱因出现发热,伴有畏寒,体温为38℃左右,以下午及夜间发热为主,无鼻塞、流涕、咽喉部不适、咳嗽、咳痰,体温可自行降至正常。第2天可再次出现发热。到当地医院诊治,给予抗感染、抗病毒治疗后症状无缓解。逐渐出现     

种痘样水疱病样皮肤T细胞淋巴瘤1例

<正>种痘样水疱病样皮肤T细胞淋巴瘤(hydroavacciniforme-like cutaneous T cell lymphoma)是近年来才认识和报道的极罕见的儿童皮肤T细胞淋巴瘤,皮疹极易与种痘样水疱病相混淆,确诊主要靠皮损活检和免疫组化。发病无明显性别差异,亚洲和拉丁美洲报道较多,1986年Oono等首次报道,国内陆续有少量该病例报道。笔者曾诊治1例,现报道如下。     

Effect of a Gluten-free Diet on the Risk of Enteropathy-associated T-cell Lymphoma in Celiac Disease

Patients with celiac disease have an increased rate of enteropathy-associated T-cell lymphoma, but conflicting data are available about the protective role of a gluten-free diet with regard to the development of this malignancy. We followed 1,757 celiac patients for a total period of 31,801 person-years, collecting data about the frequency of gluten intake and the incidence of the enteropathy-associated T-cell lymphoma. Out of the nine celiac patients who developed an intestinal lymphoma [standard morbidity ratio of 6.42 (95% CI = 2.9–12.2; P < 0.001)], only two kept a strict gluten-free diet after the diagnosis of celiac diasese and developed the malignancy after the peridiagnosis period of 3 years, dropping therefore the standard morbidity ratio to 0.22 (95%CI = 0.02–0.88; P < 0.001). The risk of developing an intestinal lymphoma for the celiac patients that used to have dietary gluten was significant (X² = 4.8 P = 0.01). These results show that a strict gluten-free diet is protective towards the development of enteropathy-associated T-cell lymphoma. Collaborating Centers Cattedra di Medicina Interna II, Univ. Cattolica, Roma, G.Gasbarrini, M.D., V. De Vitis, M.D.; Dipartimento di Pediatria, Univ. Federico II, Napoli, L. Greco, M.D., S. Auricchio, M.D.; Ospedale per gli infermi di Faenza-Azienda USL di Ravenna, D.Santini, M.D., F. Scaggiante M., M.D., Vincenzi M., Federici M.D.; Istituto Giannina Gaslini, Pediatria III, Genova, E. Castellano, M.D., A.Calvi, M.D.; Cattedra di Gastroenterologia, Dipartimento di Medicina Interna, Univ. Torino, Sategna-Guidetti, S. Grosso; Unità di Gastroenterologia, IRCCS Policlinico S. Matteo, Univ. Pavia, Campanella J., M.D., Corazza G.R., M.D.; Unita’ Operativa di Nutrizione Clinica, Ospedale S. Eugenio, Roma, G. Sandri, M.D., G. Giorgetti, M.D.; Monica Amici, Dipartimento di Medicina Interna, Policlinico S. Orsola-Malpighi, Bologna, U. Volta, M.D., C. Parisi, M.D.; Servizio di Gastroenterologia ed Endoscopia Digestiva, Ospedale USL 9, Treviso, S. Lo Perfido, M.D., ; Divisione di Gastroenterologia, IRCCS Casa Sollievo Sofferenza, San Giovanni Rotondo, Foggia, F. Perri, M.D., V. Festa M.D.,; Clinica di Gastroenterologia ed Epatologia, Univ. Perugia, M.A. Pelli, M.D., M.L. Cavalletti M.D.,; Unità Operativa di Gastroenterologia ed Endoscopia Digestiva, Ospedale di Circolo e Fondazione Macchi, Varese, S. Segato, M.D., M. Curzio M.D.,; Servizio di Gastroenterologia ed Endoscopia Digestiva, Dipartimento Oncologia, Ospedale S. Giovanni AS, Torino, M. Pennazio M.D.,, F.P. Rossini, M.D.; Cattedra di Gastroenterologia, Ist. di Clinica Medica II, Univ. La Sapienza, Roma, A. Picarelli M.D.; Divisione di Gastroenterologia, Ospedale Mauriziano Umberto I, Torino, A. Pera, M.D., E. Ercole M.D.; Unità Operativa di Gastroenterologia, Dip.to di Patofisiologia Clinica, Univ. Pol. “Careggi” Firenze, M.T. Passaleva; Clinica Pediatrica, Servizio di Gastroenterologia, Univ. La Sapienza, Roma, M. Barbato M.D.,; Istituto di Medicina Interna, Univ. Cagliari, P. Usai M.D.,, M.F. Dore M.D.,; Divisione di Gastroenterologia, Ospedale Regionale, Bolzano, F.Chilovi, M.D., L. Piazzi, M.D. L.Zancanella M.D., and Servizio di Gastroenterologia Pediatrica e Servizio di Gastroenterologia, Univ. Modena, V. Boarino, M.D., A. Ferrari M.D.     

A case of colon perforation due to enteropathy-associated T-cell lymphoma

Enteropathy-associated T-cell lymphoma(EATL) is an extremely rare disease,which is often related to glutensensitive enteropathy.It is an uncommon intestinal lymphoma with very poor prognosis and high mortality rate.In the absence of specific symptoms or radiological findings,it is difficult to diagnose early.Major complications of EATL have been known as intestinal perforation or obstruction,and only 5 cases of EATL are reported in South Korea.In this study,we report a case of 71-year-old male with symptoms of diarrhea,which later it progressed into cancer perforation of the colon.The initial colonoscopic findings were normal and computed tomography scan demonstrated a segmental wall thickening of the distal ascending colon with nonspecific multiple small lymphnodes,along the ileocolic vessels,but no signs of mass or obstruction.The histologic findings of resected specimen confirmed EATL type Ⅱ.Patient expired two weeks after the operation.Therefore,we emphasize the need of random biopsy in the presence of normal mucosa appearance on colonoscopy for the early diagnosis of EATL.     

Synchronous adenocarcinoma and extranodal natural killer/T-cell lymphoma of the colon:A case report and literature review

Extranodal natural killer/T-cell lymphoma(ENKTL) is a distinct subtype of non-Hodgkin’s lymphoma and is rare in the colon.Synchronous adenocarcinoma and ENKTL of the colon has not been reported in the literature.In the present study,we report a 63-year-old male who suffered from intermittent bloody stools for 2 mo.He did not have fever,body weight loss or night sweat.Endoscopic and imaging studies revealed a 4.5-cm ulcerative mass in the ascending colon and a 3.0-cm polypoid,easy bleeding mass in the sigmoid colon,respectively.Thought to have double carcinoma of the colon,he received simultaneous right hemicolectomy and sigmoidectomy.The pathological diagnosis was a synchronous ENKTL(ascending colon) and adenocarcinoma(sigmoid colon).The literature on synchronous adenocarcinoma and malignant lymphoma of the colon was also reviewed.     

初诊为咳嗽变异性哮喘的血管免疫母细胞性T细胞淋巴瘤一例及文献复习

目的 介绍1例初诊考虑为咳嗽变异性哮喘的血管免疫母细胞性T细胞淋巴瘤并复习相关文献,以提高临床医师对该病的认识.方法 对1例确诊为血管免疫母细胞性T细胞淋巴瘤的患者诊治过程进行分析,结合文献讨论其临床特点、诊断及治疗,并分析其误诊原因.结果 血管免疫母细胞性T细胞淋巴瘤是一种来源于滤泡辅助T细胞的非霍奇金淋巴瘤,发病率低,以中老年患者为多见,常以淋巴结肿大起病,可伴有发热等全身非特异性症状.淋巴结活检是确诊的重要依据.目前尚无统一的治疗方案,临床主要选用CHOP方案化疗.结论 血管免疫母细胞性T细胞淋巴瘤常见多发淋巴结肿大,淋巴结压迫相邻气管、支气管可出现咳嗽等症状.系统查体有助于降低误诊、漏诊可能性.     

初诊为咳嗽变异性哮喘的血管免疫母细胞性T细胞淋巴瘤一例及文献复习

目的介绍1例初诊考虑为咳嗽变异性哮喘的血管免疫母细胞性T细胞淋巴瘤并复习相关文献,以提高临床医师对该病的认识。方法对1例确诊为血管免疫母细胞性T细胞淋巴瘤的患者诊治过程进行分析,结合文献讨论其临床特点、诊断及治疗,并分析其误诊原因。结果血管免疫母细胞性T细胞淋巴瘤是一种来源于滤泡辅助T细胞的非霍奇金淋巴瘤,发病率低,以中老年患者为多见,常以淋巴结肿大起病,可伴有发热等全身非特异性症状。淋巴结活检是确诊的重要依据。目前尚无统一的治疗方案,临床主要选用CHOP方案化疗。结论血管免疫母细胞性T细胞淋巴瘤常见多发淋巴结肿大,淋巴结压迫相邻气管、支气管可出现咳嗽等症状。系统查体有助于降低误诊、漏诊可能性。     

An unusual enteropathy-associated T-cell lymphoma with MYC translocation arising in a Japanese patient: a case report

Enteropathy-associated T-cell lymphoma (EATL) is a rare peripheral T-cell lymphoma classified into 2 types, with or without celiac disease, based on histology. Type 2 EATL is less commonly associated with celiac disease, in which cells are characterized by being monomorphic and small- to medium-sized. Cells are characterized by CD8 and CD56 expression and c-MYC oncogene locus gain. We present an atypical case of type 2 EATL in the jejunum, with human T-lymphotropic virus-1 that was CD4- CD8+ CD56- CD30- CD25- TIA-1+ and granzyme B+ on immunohistological staining. It also displayed translocation of chromosome 8p24 (c-MYC), as determined by fluorescent in situ hybridization. Mucosal spreading and intraepithelial invasion by lymphoma with villous atrophy were detected adjacent to the mucosal layer. The lymphoma may be derived from intraepithelial CD8+ T cells, similar to celiac disease.     

Disappointing outcome of autologous stem cell transplantation for enteropathy-associated T-cell lymphoma

BACKGROUND: Despite treatment, enteropathy-associated T-cell lymphoma has a very poor outcome. Chemotherapy can be complicated by small bowel perforation, gastrointestinal bleeding and development of enterocolic fistulae. Here we report on the feasibility, safety and efficacy of high-dose chemotherapy followed by autologous stem cell transplantation in patients with enteropathy-associated T-cell lymphoma (three upfront and one at relapse), with or without prior partial small bowel resection. METHODS: Four patients [two males, two females, mean age 65 years (range 60-69 years)] received high-dose chemotherapy followed by autologous stem cell transplantation. Partial small bowel resection has been performed in three patients. RESULTS: All four patients completed the mobilization and leucopheresis procedures successfully and subsequently received conditioning chemotherapy and transplantation. Engraftment occurred in all patients. No major non-haematological toxicity or transplantation-related mortality was observed. One patient has ongoing complete remission 32 months after transplantation. Three patients died from relapse within few months after autologous stem cell transplantation. CONCLUSIONS: Autologous stem cell transplantation seems unsatisfactory for patients with enteropathy-associated T-cell lymphoma. More intensive conditioning and aggressive chemotherapy with/or without targeted immunotherapy as well as allogenous stem cell transplantation needs to be explored.     

Intestinal T-cell lymphomas:A retrospective analysis of 68 cases in China

AIM:To investigate the clinical features,diagnosis,treatment and prognosis of intestinal T-cell lymphomas(ITCL)by retrospective analysis.METHODS:Sixty-eight patients who were diagnosed with ITCL in case reports in the Chinese literature were compiled and reviewed.Age,gender,CD56 expression,surgical management,multifocal nature,perforation and cyclophosphamide chemotherapy were analyzed as the prognostic factors.The Kaplan-Meier method was adopted for the univariate analysis and the cumulative survival curve analysis.RESULTS:The male-to-female ratio was 1.52 to 1.The median age was 41.7 years.Twenty-seven patients had symptoms of abdominal pain or diarrhea.Thirty-six of60 patients with temperature records had high fevers at the onset of the illness.Twenty-six of 34 patients who underwent fiberoptic colonoscopy were misdiagnosed with Crohn’s disease,intestinal tuberculosis or cancer.Sixty-one patients underwent surgery.Twelve of 61 surgical patients required a second operation for anastomotic leakage or secondary perforation.The sites of lesion involvement were the jejunum(8.82%),ileum(29.41%),ileum and colon(4.41%),colon(55.88%)and appendix(1.47%).The median cumulative survival rate was 3 mo(3.00±0.48).CONCLUSION:Efforts should be made to correctly diagnose ITCL and select the proper operative approach that may reduce serious complications and create opportunities for further treatment.