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.     

胃原发性肠病相关型T细胞淋巴瘤1例报道

本文报道1例胃原发性肠病相关型T细胞淋巴瘤。患者为1例22岁年轻女性,初始临床分期为Ⅳ期,首先采取了CHOP、GDP、HD-MTX、IEO、DHAP方案序贯化疗,然后行远端胃切除术与淋巴结清扫术,为获得较长的生存期还进行了局部姑息性放疗。治疗取得了良好的疗效,患者的生存期已超过2年,并且生活质量较高。本病例强调了在高侵袭性结外淋巴瘤的治疗中外科手段的重要性。     

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…     

肺部结外NK/T细胞淋巴瘤1例并文献复习

<正>结外NK/T 细胞淋巴瘤(extranodal NK/T-cell lymphoma, ENKTL)是非霍奇金淋巴瘤的一种亚型,临床很罕见,且恶性程度高,进展快,预后很差。可累及鼻腔、上呼吸道、皮肤、胃肠道、大脑、睾丸等结外部位,累及肺部的ENKTL更为少见。肺部ENKTL临床及影像学表现缺乏特异性,在早期很容易被误诊为肺炎。本文报道2020年10月在我院确诊的一例肺部ENKTL,并对其临床表现、胸部CT特点、病理特征和预后进行了描述。     

首发于肺的T细胞恶性淋巴瘤一例

患者男性 ,37岁 ,木工。因反复咳嗽、咯血两月余 ,于2 0 0 1年 3月 2 7日入院。患者 2 0 0 1年 1月份因感冒出现咳嗽、咳痰 ,痰中带血丝 ,胸部Ｘ线片示左肺炎症 ,予青霉素抗炎治疗 ,效果不佳 ,且痰中带血丝较前增多 ,时而咯出血块 ,复查胸片示肺部病变扩展 ,于 3月 5日去外院就诊 ,拟诊为过敏性肺炎 ,并予以红霉素药物治疗 ,症状未缓解。 1周后转入某大医院就诊 ,纤维支气管镜 (纤支镜 )刷检出白色念珠菌 ,予以大扶康、大蒜素治疗十天 ,在药物治疗期间 ,咳嗽加重 ,并出现高热 ,达 39℃以上 ,尤以夜间为甚 ,停药后体温反而降至 38℃左右。痰…     

皮下脂膜炎样T细胞淋巴瘤2例报告并文献复习

目的：探讨皮下脂膜炎样T细胞淋巴瘤（SPTCL）的临床和病理特征。方法：对2例SPTCL的临床资料进行分析，并结合文献复习，为其诊断和鉴别诊断提供一条思路。结果：临床上首发症状均表现为皮下结节，单发或多发。全身表现多种多样，但均出现持续高热、消瘦、肝功能严重受损，累及骨髓，伴致死性嗜血细胞综合征，病程进展迅速，生存不超过1年；病理检查见皮下脂肪组织内有原发的小、中或大的多形性T细胞，围绕脂肪细胞呈花环状外观。周围区组织细胞反应性增生活跃，伴有吞噬红细胞现象，并见多核巨细胞和肉芽肿样反应。瘤细胞浸润在脂肪小叶内，而脂肪小叶间隙无累及。肿瘤表达细胞毒T细胞的免疫表现。结论：SPTCL是一种以累及皮下脂肪组织为主的特殊类型的淋巴瘤，伴嗜血细胞综合征者预后差。     

以胸部症状首发的血管免疫母细胞性T细胞淋巴瘤一例报告并文献复习

血管免疫母细胞性T细胞淋巴瘤(AITL)是少见的侵袭性外周T细胞淋巴瘤,主要的临床表现多变,累及全身淋巴结、肝、脾等部位,常有发热、皮疹、体质量下降,大部分患者伴有免疫功能异常,临床表现复杂多变。本研究通过对以胸腔积液、双肺多发渗出影为首发临床表现的AITL患者的各项辅助检查结果、诊疗经过进行回顾并复习相关文献,旨在提高临床医师对AITL的认识,选择合适的检查措施来提高诊断的阳性率。     

以肺部症状为首发的淋巴瘤39例临床分析

目的探讨以肺部症状为首发的淋巴瘤的临床特点、诊断方法。方法对39例以肺部症状首发的淋巴瘤的临床表现,影像学特点,诊断方法进行分析。结果以肺部症状为首发的淋巴瘤较为少见,临床表现及影像学特征均无特异性。结论重视查体,及时行相关的辅助检查有利于淋巴瘤的诊断。     

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.     

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.     

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.     

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。     

以高热、肺部空洞为表现的肺淋巴瘤1例及文献复习

目的探讨肺淋巴瘤的临床特征。方法回顾性分析1例以高热、肺部空洞为表现的肺淋巴瘤患者的临床资料,并复习相关文献。结果以高热、肺部空洞为主要表现的肺淋巴瘤罕见,诊断主要靠病理。结论临床医师应提高对肺淋巴瘤的认识,减少误诊。     

A case of intestinal T-cell lymphoma with repeated episodes of perforation

A case of intestinal cytotoxic/suppressor T-cell lymphoma with repeated episodes of perforation was seen in a 77-year-old Japanese male who was admitted complaining of severe abdominal pain accompanied by high grade fever. Surgery revealed diffuse peritonitis due to perforation in the sigmoid colon. After a loop sigmoid colostomy the patient recovered to somedegree. Seven days later, however, another perforation occurred. Several aneurysmal swellings accompanied by perforation in the small intestine, forming a mass of 5 X 5 cm, were seen. Although the patient tolerated this operation, generalized emaciation proceeded and the patient died 10 days late. Histological examination at the second operation showed diffuse medium-sized lymphoma of the Lymphoma Study Group (LSG) classification infiltrating the entire depth of the intestinal wall. Destruction of muscle cells was prominent. An immunohistochemical study using fresh frozen material was positive for CD3, and CD8. This marked destruction of the muscle layer by lymphoma cells may be associated with repeated episodes of perforation.     

Bilateral pulmonary nodules as a manifestation of primary pulmonary T-cell lymphoma

Primary pulmonary lymphoma (PPL) is an uncommon pathology and is usually of the B-cell type, originating in lymphoid tissue associated with the bronchial mucosa (MALT/BALT lymphoma). Very few cases of T-cell PPL, the majority diagnosed by open lung biopsy, have been described in medical literature. We report a case of an immunocompetent patient with fever and bilateral pulmonary nodules, diagnosed with T-cell PPL by transbronchial biopsy. The patient’s condition deteriorated and she responded poorly to chemotherapy. PPL should be included in the differential diagnosis of patients with fever and bilateral pulmonary nodules.