血管免疫母细胞性T细胞淋巴瘤临床病理研究进展

血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T-cell lymphoma,AILT)是外周T细胞淋巴瘤中比较常见的一种类型,四川大学华西医院病理科从1990年至今诊断AILT已超过200例.相对于已研究较透彻的B细胞淋巴瘤而言,我们对AILT知之甚少,导致治疗的无奈和极差的预后.在AILT的研究中,如果把认识到其为一真正的淋巴瘤而非一种瘤前病变看作第一个里程碑,近年来关于CXCL13抗体在AILT中的特异性表达无疑是第二个里程碑,它不仅阐明了AILT的来源而且将肿瘤细胞从纷杂的背景中标记出来,为今后的相关研究奠定了一个重要的基础.本文对AILT的命名及发病率、临床表现、组织学特点、免疫表型、遗传学改变、鉴别诊断、治疗及预后等方面的问题进行综述.     

血管免疫母细胞淋巴腺病样T细胞淋巴瘤1例

血管免疫母细胞淋巴腺病样Ｔ细胞淋巴瘤１例关键词血管免疫母细胞淋巴腺病，淋巴瘤溶血性贫血血管免疫母细胞淋巴腺病（ａｎｇｉｏｉｍｍｕｎｏｂｌａｓｔｉｃｌｙｍｐｈａｄｅｎｏｐａｔｈｙ，ＡＩＬＤ）是一种最近报道的具有充分临床病理特征性疾病。２０％病例转变为恶...     

血管免疫母细胞性淋巴结病15例临床病理分析

分析１５例血管免疫母细胞性淋巴结病，认为小剂量强的松可用于诊断性治疗；提出将该病分为Ｂ免疫母细胞型、Ｔ免疫母细胞型及Ｂ和Ｔ免疫母细胞混合型３个组织学亚型；认为该病预后可能与诊断早晚、治疗用药是否恰当及时有关。     

血管免疫母细胞性淋巴结病及血管免疫母细胞性T细胞淋巴瘤

为了深入调查血管免疫母细胞性淋巴结病（ＡＩＬＤ）和血管免疫母细胞性Ｔ细胞淋巴瘤（ＡＩＴＬ）的临床病理、病变性质诸方面，采用了免疫组化和聚合酶链反应（ＰＣＲ）等方法对５例进行了分析。该５例患者均有全身淋巴结肿大（但直径多小于１ｃｍ），肝脾肿大、发热和多项血液指标改变。ＡＩＴＬ时在ＡＩＬＤ病变背景上出现异型的透明Ｔ细胞浸润为诊断和鉴别依据。５例中４例行ＴＣＲ－β重排分析均呈现克隆性改变。并对４例埃泼斯坦－巴尔病毒（ＥＢＶ）感染情况进行分析，其中３例ＥＢＶＤＮＡ阳性。认为ＡＩＬＤ可能是一种与ＥＢＶ感染相关的瘤前病变，发展为淋巴瘤的比例较高。     

伴有RS细胞样细胞的血管免疫母细胞性T细胞淋巴瘤一例

患者男,70岁。不明原因腹胀2个月余,不易缓解,无明显腹痛,无发热及胸闷。于当地医院行胸腹CT发现左肺下叶感染并左侧胸腔大量积液,纵隔淋巴结稍大,脾大及腹腊后淋巴结广泛肿大,     

血管免疫母细胞性T细胞淋巴瘤侵犯骨髓相关病理特征分析

目的 探讨血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T cell lymphoma, AITL)侵犯骨髓的病理特征。方法 回顾性分析32例AITL侵犯骨髓的临床病理特征,采用免疫组化EnVision法和流式细胞术检测AITL相关免疫标记,通过T系基因重排分析T细胞克隆性。结果 肿瘤细胞浸润模式主要以结节状(20/32,62.5%)、间质性或小簇状(10/32,31.3%)为主,结节成分较杂,可呈“肉芽肿样改变”;肿瘤细胞主要为小至中等大小淋巴细胞,异型不明显,少数病例可出现明显浆细胞增生。19例行免疫组化染色,CD4阳性T细胞较少,平均为8.4%;滤泡辅助T细胞(T follicular helper cells, TFH)相关免疫组化标记阳性率分别为：CD10(7/14,50.0%)、BCL6(6/19,31.6%)、PD-1(13/19,68.4%)、CXCL13(13/19,68.4%),大部分病例肿瘤细胞PD-1和CXCL13同时阳性,且阳性细胞数量较少(均<1%)。24例行流式细胞术检测,其中22例均一致性表达胞质CD3(cCD3)、CD5、C...     

27例血管免疫母细胞性T细胞淋巴瘤的免疫表型分析

目的：分析27例血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T-cell lymphoma,AITL)的免疫表型,重点探讨CXCL-13、PD-1、CD10、bcl-6在其诊断中的作用,其中3例CD30阳性病理的诊断意义及问题。方法：收集黄石市第二医院病理科2009年1月至2015年7月间诊断的27例血管免疫母细胞性T细胞淋巴瘤,对其进行常规形态学和免疫学表型分析,观察对预后的影响。结果：27例血管免疫母细胞性T细胞淋巴瘤中,免疫组织化学表达阳性：CD3(27/27)、CXCL13(27/27)、PD-1(27/27)、CD10(20/27)、bcl-6(23/27)其中有3例CD30散在阳性(3/27),CD20(0/27),CD4和CD8阳性细胞混合组中,20例CD4阳性细胞占优势,5例CD8阳性细胞占优势,1例CD4、CD8全阴性,1例CD4、CD8全阳性。CD21滤泡树突细胞阳性,并显示明显的围绕血管特征,同时也提示在肿瘤中均存在滤泡树突网。结论：AITL是一种来源生发中心辅助性T细胞的高度侵袭性肿瘤。CXCL13PD-1CD10bcl-6在诊断中为有用标记物,四种联合应用更能提示诊断。本组3例AITL中存在CD30阳性细胞除鉴别诊断外,其临床意义有待研究。     

血管免疫母细胞性淋巴结病/淋巴瘤的回顾性分析

目的 探讨血管免疫母细胞性淋巴结病(AILD)和血管免疫母细胞性淋巴瘤(AITL)的临床及病理学特征，以及p53蛋白在AILD和AITL中表达的临床意义。方法 对原诊断AILD 9例阳AITL 9例重新复查病理切片及临床资料．采用免疫组织化学SP法，对复验后的病例进行免疫表型标记及p53蛋白检测。结果 AILD的误诊率5/9、AITL的误诊率5／9。AILD 4例中p53阳性表达1例，AITL 7例中p53阳性表达5例。结论 误诊的最主要原因是对AILD和AITL的诊断标准掌握不够准确，它们与一些有类似形态结构肿瘤的主要区别在于有高度增生的分支状小血管，呈弥漫性分布．在AILD和AITL的鉴别诊断中，p53可作为一个重要的免疫学指标。     

血管免疫母细胞性淋巴结病的新认识

血管免疫母细胞性淋巴结病的新认识刘强，刘振延１９７４年Ｆｒｉｚｚｅｒａ等［１］报道伴异常蛋白血症的血管免疫母细胞性淋巴结病（ａｎｇｉｏｉｍｍｕｎｏｂｌａｓｔｉｃｌｙｍｐｈａｄｅｎｏｐａｔｈｙｗｉｔｈｄｙｓｐｒｏｔｅｉｎｅｍｉａ，ＡＩＬＤ）为命名的一类...     

淋巴结血管内T细胞淋巴瘤1例报道及文献复习

目的　探讨血管内淋巴瘤 (IVL)的临床病理特征。方法　对 1例腹股沟淋巴结IVL临床、病理组织学及免疫表型进行观察分析并复习文献。结果　男性 31岁 ,不明原因高热伴消瘦 5 0天 ,右腹股沟直径 1cm淋巴结 1枚 ,B超示肝脏轻度增大 ,血LDH明显升高伴ESR及转氨酶轻度升高 ,外周血WBC 3 3× 10 7/L ,骨髓像、多种病原及各肿瘤相关抗原检测均无异常。病理活检 :腹股沟淋巴结大部分破坏 ,代之以大量扩张的中小血管 ,腔内充满大量异型淋巴样细胞 ,局部伴管壁、管周浸润并累及结外脂肪组织。瘤细胞免疫表型CD4 5、CD4 5RO、CD3阳性 ,CK、CD6 8、CD79α、CD2 0均阴性 ,血管壁及内皮细胞CD31、CD34阳性。行CHOP化疗后症状缓解 ,现仍在随访中。结论　IVL是一罕见的非霍奇金淋巴瘤 ,好发于中枢神经系统及皮肤 ,其他部位少见 ,绝大数为B细胞型 ,T型罕见 ,以浅表淋巴结活检确诊者尚无报道。临床表现有一定提示性 ,确诊靠组织病理学检查 ,部分病例对化疗敏感 ,但多数病例预后差     

Distinguishing angioimmunoblastic T-cell lymphoma from peripheral T-cell lymphoma, unspecified, using morphology, immunophenotype and molecular genetics

AIMS: To identify distinguishing histological, immunophenotypic and molecular genetic features between angioimmunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphoma (PTL). METHODS: Nodal T-cell lymphomas examined (n =137), included AITL (n = 89), PTL (n = 22), anaplastic large cell lymphoma (n = 16) and 'AITL/PTL indeterminate' (n = 10) with overlapping features between AITL and PTL, showing morphology typical of AITL but lacking follicular dendritic cell expansion. Immunohistochemistry for CD3, CD20, CD21 and CD10, in situ hybridization for Epstein-Barr virus encoded RNA (EBER) and polymerase chain reaction for T-cell and B-cell clonality analysis were performed. RESULTS: Of the AITLs, 74/89 showed typical morphology, whereas 15/89 showed hyperplastic follicles. AITL and 'AITL/PTL indeterminate' showed a polymorphous infiltrate and prominent vascularity in all cases. In both groups, CD10 was present in the majority and clear cells and EBER positivity were specific (but not universal) features lacking in PTL. Detection of T-cell clonality was significantly higher in AITL (90%) compared with PTLu (59%). CONCLUSION: Clear cells and EBV infection (when present) are useful distinguishing features and CD10 a sensitive and specific marker of AITL. Hyperplastic follicles are present in a significant minority of AITL. AITL/PTL indeterminate probably falls within the spectrum of AITL rather than PTL.     

肾脏原发性淋巴瘤临床病理分析

目的 :对肾脏原发性淋巴瘤的临床病理特点、组织学起源、诊断及鉴别诊断等进行初步探讨。方法 :对 1例手术切除的肾脏原发性淋巴瘤标本做HE染色和S P免疫组化染色 ,光镜观察。结果 :左肾脏上极见一 7 5cm× 6cm× 4cm界限不清的肿块。镜下见在肾实质内有弥漫大片淋巴瘤细胞浸润。瘤细胞核呈略不规则形 ,染色质呈凝块状。免疫表型肿瘤细胞表达LCA、L2 6、IgA弥漫阳性。病理诊断为弥漫小核裂细胞型。 结论 :肾脏原发性淋巴瘤甚为罕见。结合文献 ,其主要诊断依据为 :①肾脏内有弥漫大片形态一致的淋巴瘤细胞浸润 ;②肿瘤主要位于肾脏实质内 ,肾包膜及其周围脂肪组织内亦可见瘤细胞浸润 ;③患者浅表淋巴结不肿大 ,CT检查未见胸、腹腔内有肿大的淋巴结 ;④骨髓穿刺涂片和活检未见异常细胞。⑤发现肾脏淋巴瘤至少 3月后未发现其它部位的淋巴瘤。发生于肾脏的淋巴瘤应与肾脏的肉瘤样癌、Wilm瘤、慢性炎症等相鉴别。其主要治疗方法为肾切除加化疗和 (或 )放疗。     

Development of angioimmunoblastic T-cell lymphoma after treatment of diffuse large B-cell lymphoma: a case report and review of literature

Cases of diffuse large B-cell lymphoma (DLBCL) arising after the initial diagnosis of angioimmunoblastic T-cell lymphoma (AITL) and DLBCL synchronous with AITL have been reported. To date, there is no report on the subsequent development of AITL in patients with DLBCL. Here we presented a rare case of AITL developing six months after the initial diagnosis of DLBCL. In order to investigate the clinical and molecular features of patients with AITL and DLBCL, we also reviewed the literature on AITL patients developing DLBCL, and patients with composite AITL and DLBCL.     

Clinicopathological features of primary hepatic diffuse large B-cell lymphoma: a report of seven cases and a literature review

We studied the imaging and histopathological features of primary hepatic diffuse large B-cell lymphoma in order to explore the clinicopathological features, diagnosis, differential diagnoses, and treatment. Immunolabelling was performed in seven cases of primary hepatic diffuse large B-cell lymphoma using histological and immunohistochemical techniques. The clinical manifestations; imaging, histopathological, and immunohistochemical features; treatment; and prognosis of primary hepatic diffuse large B-cell lymphoma were observed and analyzed in light of the relevant literature. The average age of the seven patients was 63.4 years. Moreover, bulge of the upper right abdomen and progressive athrepsia and anemia were observed in all seven patients. Computed tomography (CT) revealed the presence of multiple solid hypodense lesions. Further, CT also revealed an enhanced irregular focus. Histopathological analysis revealed the following characteristics: heavy infiltration composed mainly of medium-sized round cells with a lightly stained cytoplasm, prominent nucleoli and vesicular nuclei, nuclear fission and visible sky star phenomena. The tumor cells showed diffuse expression of CD19, CD20, and CD79a, with the percentage of Ki67-positive cells being 75%-80%. All these findings indicated that primary hepatic diffuse large B-cell lymphoma is rare and generally has a poor prognosis. Biopsy and immunohistochemical staining are helpful in its diagnosis. Further, the differential diagnoses include secondary liver diffuse large B-cell lymphoma, low/undifferentiated cancer of the liver, hepatoblastoma, leukemia of the liver, and other tumors. Early surgery and chemotherapy can have a good curative effect.     

Sequential development of diffuse large B-cell lymphoma in a patient with angioimmunoblastic T-cell lymphoma

Lymphoma of different histologic type can occur in the same patient. Here, we describe a 64-year-old male patient with angioimmunoblastic T-cell lymphoma (AITL) who subsequently developed diffuse large B-cell lymphoma (DLBCL). At the time of initial diagnosis, histologic examination of a left inguinal lymph node of the patient and a monoclonal pattern of TCRβ gene rearrangement showed typical features of AITL, and there was no evidence of a monoclonal B-cell population. Twenty-six months later, he had generalized lymphadenopathy and organs involvement by DLBCL. A monoclonal IgH gene rearrangement proved de novo development of secondary B-cell lymphoma and excluded relapse of a primary composite lymphoma. The in situ hybridization analysis showed Epstein-Barr-encoded RNA (EBER) sporadic positivity in sample collected from AITL but extensive positivity in the immunoblasts collected from DLBCL. Our observation supports the hypothesis that Epstein-Barr virus (EBV) is etiologically related to AITL in this case. Clonal expansion of EBV-associated DLBCL is a secondary event in AITL via EBV infection or reactivation.     

淋巴瘤样肉芽肿型大B细胞淋巴瘤

目的 探讨淋巴瘤样肉芽肿型大B细胞淋巴瘤(原名淋巴瘤样肉芽肿病，lymphomatoid granulomatosis，LYG)的病理形态特征、病变性质及鉴别诊断要点。方法 对l例LYG的组织形态学、免疫组织化学、EBV原位分子杂交(EBER)结合临床特征进行了分析。结果 l例63岁男性患者，临床上表现为多系统多器官性病变，主要累及肺，表现为双肺内境界清楚的圆形结节，呈孤立性或弥漫性分布，并出现多发性皮下结节，发热、体重减轻、全身无力等症状。皮下结节活检示多个肉芽肿样结构，细胞形态多样，见组织样细胞、非典型淋巴样细胞、小淋巴细胞、浆细胞及散在多核巨细胞，可见一血管壁有淋巴样细胞浸润，未见明显中心粒细胞，可见核分裂象。肺部穿刺组织示弥漫淋巴样细胞浸润，并见灶性坏死，免疫表型示瘤细胞呈CD20 ，CD79α ，CD43 ,CD3-,GraB-，EBV散在 ，CK-，Syn-，原位杂交示EBER 。结论 本例LYG是一种罕见的淋巴瘤样肉芽肿型大B细胞性淋巴瘤，与EBV相关。临床与影像学上与Wegener肉芽肿相似。肺部出现结节状病灶时，临床上易与结核、肉芽肿病、肺癌及炎性假瘤等相混淆，病理上须与结核、非特异性肉芽肿病、结外的外周T细胞淋巴瘤等相鉴别，形态学、免疫表型结合临床特征可明确诊断。     

血管内淋巴瘤

目的：探讨血管内淋巴瘤的临床病理特征。方法：对1例血管内淋巴瘤进行免疫表型分析及原位杂交检测与EB病毒的关系,并文献复习,结果：女性,48岁,不明原因发热伴体重下降3个月,CT扫描示子宫肿块而行子宫加双侧附件切除,病理学检查发现瘤细胞位于血管内,伴少许管周浸润,瘤细胞免疫表型CD45（LCA）和CD20（L26）呈阳性表达,原位杂交显示E－BERs阴性,患者经CHOP联合化疗后获得完全缓解,结论：血管内淋巴瘤是一种极罕见的B细胞肿瘤,此瘤的诊断只能依赖病理学检查,治疗上应予以积极联合化疗。     

Progressive and painful wound as a feature of subcutaneous panniculitis-like T-cell lymphoma (SPTCL): report of a case and review of literature

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon extranodal non-Hodgkin lymphoma, with an aggressive course with no well-defined treatment. This article describes a 56-year-old man, treated surgically 7 months earlier for a subcutaneous nodosity near the left axilla, presenting with a progressive inflamed wound, pain, and high fever (39°C). Treatment with systemic antibiotics and topical anti-inflammatory dressings failed. After 7 months, the patient was diagnosed with SPTCL based on biopsy results and a multidisciplinary consultation. While undergoing systemic chemotherapy with corticosteroid therapy, his wound become more painful, larger, and covered with necrotic tissue. Fifty days after chemotherapy with corticosteroid therapy, his wound became seriously painful and increasingly necrotic. He developed a serious stomachache and abdominal distension, rapidly became comatose, and died. The aim of this case report is to present our experience of the different clinical signs of SPTCL to expedite its early diagnosis in future. We summarize the main clinical characteristics of SPTCL as a rapidly progressing and increasingly painful wound with necrotic tissue, involving a multisystem disorder, which is easily misdiagnosed, responds poorly to corticosteroid and chemotherapy treatments, and has a high mortality rate. The pathological characteristics are early inflammation, advancing to profuse infiltration of the subcutaneous adipose tissues by CD3⁺ and/or CD8⁺ T-cell lymphoma cells. Clinicians must cooperate with pathologists and oncologists to diagnose this disease as soon as possible and to avoid a misdiagnosis. The use of antibiotic and painkillers should minimize the patient’s discomfort and control rapid wound development. Future studies are required to investigate the optimal wound treatment and whether the necrotic tissue should be removed.     

脊索瘤样脑膜瘤1例并文献复习

目的:探讨脊索瘤样脑膜瘤(chordoid meningiomas,CM)的临床与病理特点.方法:应用组织病理学、组织化学以及免疫组织化学方法对1例CM进行观察,同时复习相关文献进行讨论.结果:CM病理特点为黏液样基质中有呈分叶状或条索状排列的肿瘤细胞,细胞质呈淡染或嗜酸性染色,部分细胞呈液滴状,分布于黏液样基质中,类似脊索瘤样细胞;肿瘤细胞中亦可见典型脑膜上皮细胞区域.组织化学染色阿尔辛蓝-过碘酸-Schiff反应(alcian blue and peridic acid-schiff's reacyion,AB/PAS)呈阳性反应.免疫组织化学染色显示:肿瘤细胞波形蛋白(vimentin)、上皮膜抗原(epithelial membrane antigen,EMA)表达阳性,孕激素受体(progesterone receptor,PR)灶状阳性,广谱细胞角蛋白(pan cytokeratin,CKpan)、S-100钙结合蛋白(S-100 calcium binding protein,S-100)表达阴性.结论:CM是发生于颅内的较少见的脑膜瘤亚型,需要与脊索瘤、黏液性软骨肉瘤及转移性黏液腺癌等鉴别.