母细胞型套细胞淋巴瘤的临床病理观察

目的 探讨母细胞型套细胞淋巴瘤（BV-MCL）的病理特征、免疫表型、诊断和鉴别诊断。方法 对2例BV-MCL行HE和免疫组化染色观察。结果 1例为经典型母细胞型，肿瘤细胞弥漫、中等偏大、染色质细，核分裂87个／10HPF；另1例为多形型母细胞型，肿瘤细胞弥漫、多形，中等大的细胞与大细胞混杂，核染色质粗，可见明显的核仁，核分裂36个／10 HPF。2例均cyclinD1（＋）。结论 BV-MCL少见，是MCL的一种变型，其病理形态和免疫表型特殊，需要与弥漫性大B细胞淋巴瘤、前驱B淋巴母细胞白血病／淋巴瘤等鉴别。     

原发中枢神经系统的弥漫大B细胞淋巴瘤病理学诊断特征

目的探讨原发中枢神经系统的弥漫大B细胞淋巴瘤的病理学诊断特征。 方法回顾性分析2010年1月至2015年12月经泰安市中心医院病理科确诊的15例原发中枢神经系统的弥漫大B细胞淋巴瘤患者的临床资料,总结原发中枢神经系统的弥漫大B细胞淋巴瘤的病理形态学特点及免疫表型。 结果患者男性10例,女性5例,年龄52～64岁;15例患者临床表现为头痛头晕,反应迟钝或行走不稳。CT检查均发现颅内占位,4例发生了转移,分别转移到睾丸、肝脏、肺脏和乳腺,其余患者正电子发射计算机断层显像(PET-CT)检查均未见全身其他部位肿瘤。光镜下肿瘤细胞表现为弥漫性生长,特征性的分布于血管周隙;肿瘤细胞大多相似于中心母细胞,与反应性小淋巴细胞、巨噬细胞、活化的小胶质细胞以及反应性星形细胞混杂。免疫组织化学染色显示肿瘤细胞均弥漫强表达白细胞分化抗原20(CD20)及B细胞系特异性激活蛋白(PAX5),其中3例表达白细胞分化抗原10(CD10),7例均表达B细胞淋巴瘤因子6(Bcl-6)及多发性骨髓瘤致癌蛋白(MUM1),余5例均表达MUM1,增殖细胞核抗原(Ki-67)指数70%～90%。 结论原发中枢神经系统的弥漫大B细胞淋巴瘤是一种相对少见的淋巴瘤,以弥漫性分布于血管周隙的中心母细胞样细胞为特征;临床表现与其发生部位有关,掌握其共同的特征并结合免疫组织化学技术方能正确诊断。     

结外淋巴瘤病理的新进展

将1997年WHO淋巴瘤新分类与Lukes和Collins分类相比较，发现新分类突出的特点是确立了许多淋巴结以外的新瘤种，主要为：（1）边缘区结外淋巴瘤（脾、MALT型边缘区B细胞淋巴瘤）；（2）纵隔（胸腺）、血管内、原发性渗出性大B细胞淋巴瘤；（3）NK/T细胞淋巴瘤（鼻型、肠病型、脂膜炎样T细胞淋巴瘤）；（4）系统性伴结外病变（T、Null、B细胞型）及原发性皮肤间变性大细胞淋巴瘤等。新的瘤种具有各自的临床特点。病理组织学、免疫表型、分子遗传学特征与诊断要点。     

鼻咽部血管内大B细胞性淋巴瘤临床病理特征

目的探讨鼻咽部血管内大B细胞淋巴瘤(IVL)的临床病理特点及鉴别诊断。方法对1例鼻咽部血管内大B细胞淋巴瘤的临床病理学特点及免疫表型进行分析,并结合相关文献进行讨论。结果瘤细胞主要聚集于小血管和毛细血管腔内,部分可位于中等血管内。这些血管内的淋巴样细胞,体积增大,不规则,泡状核,核仁明显,核分裂象常见。免疫组化显示肿瘤细胞CD20、CD79a、LCA(+),Ki-67增殖指数很高。结论血管内大B细胞淋巴瘤是弥漫性大B细胞淋巴瘤的一种非常罕见的亚型,其临床表现无特征性,明确诊断主要依靠活检标本的病理组织学特征和免疫表型及尸检。     

一例NK样T细胞淋巴瘤/白血病--附文献复习

目的提高对NK样T细胞淋巴瘤／白血病的认识？方法报道1例NK样T细胞淋巴瘤／白血病患者，并复习文献，总结该病临床及实验室检查特点。结果患者以发热、皮肤疱疹起病，肿瘤细胞主要分布于皮下小血管周围和血管腔内，免疫表型呈CD3（＋），CD8（＋），CD4（-），CD5（-），CDl0（-），CD19（-），CD57（-），CD56（＋），穿孔素（＋）和颗粒酶B（＋），T细胞受体1重排阳性。经糖皮质激素治疗一度好转，但很快复发，进展为NK样T细胞白血病，病程1年，死于多器官功能衰竭。结论NK样T细胞淋巴瘤／白血病非常少见，具有独特的临床、组织病理学和免疫表型特征，对现有治疗不敏感，预后恶劣。     

原发性皮肤弥漫性大B细胞性淋巴瘤(腿型)17例临床病理分析

目的探讨原发性皮肤弥漫性大B细胞性淋巴瘤(腿型)(PCDLBCLLT)的临床病理特点。方法回顾性分析17例PCDLBCLLT的临床资料、组织学形态和免疫组化标记。结果 17例PCDLBCLLT的发病年龄为31～86岁,平均64.4岁;其中男性9例,女性8例,男女之比为1.1∶1;主要发生于腿部和躯干部。组织学表现为弥漫分布的肿瘤细胞,以中心母细胞和免疫母细胞为主,核分裂象易见,肿瘤组织不累及表皮。免疫组化:肿瘤细胞表达B细胞相关抗原,bcl-2、MUM1、FOX-P1和bcl-6(+),Ki-67增殖指数为60%～90%。结论 PCDLBCLLT是一种独特类型的大B细胞性淋巴瘤,预后较差。     

MUM1/IRF4在弥漫性大B细胞淋巴瘤中的表达及其意义

目的：研究MUM1（multiple myeloma oncogene 1）/IRF4（interferon regulatory factor 4）蛋白在弥漫性大B细胞淋巴瘤组织中的表达及意义。方法：应用免疫组织化学S-P法,检测82例弥漫性大B细胞淋巴瘤标本中MUM1/IRF4蛋白的表达。结果：弥漫性大B细胞淋巴瘤MUM1蛋白阳性表达率为54.9%（45/82）,其中中心母细胞型阳性表达率为50.0%（36/72）,免疫母细胞型阳性表达率为100.0%（4/4）,间变性大B细胞型阳性表达率为100.0%（4/4）,其他50%（1/2）。结论：MUM1的表达可能与细胞的活化有关。     

浆膜腔淋巴瘤21例临床病理分析

目的回顾性分析21例浆膜腔积液中细胞学诊断为淋巴瘤的临床病理学特征、免疫表型、诊断与鉴别诊断及治疗与预后。方法收集西京医院2014-01—2016-05间由浆膜腔积液诊断为淋巴瘤者21例,对其进行常规涂片、染色、离心包埋切片、免疫细胞化学染色,及对部分病例进行基因重排分析,并对照其细胞学诊断和活检组织病理诊断的符合率。结果 21例中,男性14例,女性7例,年龄2~87岁,中位年龄64岁。14例为胸腔积液,6例为腹腔积液,1例为心包积液。21例全部为非霍奇金淋巴瘤。细胞学经免疫细胞化学染色或基因重排明确分型的13例,2例淋巴浆细胞样淋巴瘤,3例弥漫性大B细胞性淋巴瘤,1例原发性渗出性淋巴瘤,2例浆母细胞淋巴瘤,1例血管免疫母细胞性淋巴瘤,1例T淋巴母细胞性淋巴瘤,1例B淋巴母细胞性淋巴瘤,2例小淋巴细胞性淋巴瘤。其余8例中4例因有淋巴瘤病史,未行免疫细胞化学染色,2例经免疫细胞化学染色证实为B细胞性淋巴瘤。结论浆膜腔积液中淋巴瘤病例较少见,临床症状不典型,易误诊为转移癌或结核性浆膜腔积液。对浆膜腔积液进行仔细的形态学观察,同时离心包埋做免疫细胞化学染色和基因重排检测可有助于诊断和分型。     

血管内大B细胞淋巴瘤4例临床病理分析

目的探讨血管内大B细胞淋巴瘤的临床和病理特点,以提高其诊治水平。方法收集并分析了4例血管内大B细胞淋巴瘤的临床特征和病理资料,用形态学方法和免疫组化染色进行研究。结果发生于皮肤2例,出现皮肤斑块和破溃;发生于脑部2例,出现神经系统症状。镜下所见相似,瘤细胞仅位于小血管腔内,瘤细胞大,核空泡状,核仁明显,核分裂易见。瘤细胞表达B细胞标记LCA( ),CD20( ),CD79a( ),CD30(-),CD3(-),CD45RO(-),Ⅷ因子,Desmin阴性。结论该瘤是一种罕见的结外弥漫性大B细胞淋巴瘤,诊断只能依靠病理检查确诊。该瘤高度恶性和高度侵袭性,对化疗不敏感,多数患者发病后短期死亡,预后很差,应早期诊断,积极联合化疗。     

155例非霍奇金淋巴瘤患者细胞遗传学分析

目的 了解非霍奇金淋巴瘤（NHL）患者染色体异常与WHO病理组织分型之间的关系，并与国外NHL染色体异常类型进行比较。方法 采用常规染色体G带分析和荧光原位杂交（FISH）方法对155例NHL患者的淋巴结组织进行细胞和分子遗传学研究。结果 155例NHL患者中常见的病理类型是弥漫大B细胞淋巴瘤（DLBCL）（59例，38．1％）、滤泡性淋巴瘤（27例，17．4％）、B小淋巴细胞淋巴瘤（16例，10．3％）、非特指周同T细胞淋巴瘤（13例，8．4％）、血管免疫母细胞性T细胞淋巴瘤（11例，7．1％）。155例NHL患者中染色体异常为119例，占76．8％。滤泡性淋巴瘤、B小淋巴细胞淋巴瘤、DLBCL、间变性大细胞淋巴瘤和前体T淋巴母细胞淋巴瘤染色体异常率较高，分圳为96．3％、87．5％、86．4％、83．3％、83．3％。DLBCL中复杂核型占86．3％，染色体结构异常累及最多的是3，6，14，1号染色体，41．2％为3q27异常，43．1％的病例有1号染色体异常。6q21、6q23和6q25异常占23．5％。DLBCL中典型t（14；18）的病例只有2例，明湿低于国外报道。用FISH方法检测DLBCL中IgH重排阳性率为40．1％。16例B小淋巴细胞淋巴瘤均未发现13q14缺失，只发现2例有13q10异常。11例血管免疫母细胞性T细胞淋巴瘤中只有3例核型异常。结论 我国淋巴瘤的病理类型分布与欧美国家有明显不同。尽管DLBCL染色体异常类型基本与国外相似，但t（14；18）较少见。与国外报道相比，B小淋巴细胞淋巴瘤和血管免疫母细胞性T细胞淋巴瘤染色体异常率较低，染色体异常类型也有差异。     

T细胞/组织细胞丰富的大B细胞淋巴瘤病理学诊断特征分析

目的探讨T细胞/组织细胞丰富的大B细胞淋巴瘤的病理诊断学特征。 方法回顾性分析12例2010年1月至2017年12月经泰安市中心医院病理科确诊的T细胞/组织细胞丰富的大B细胞淋巴瘤患者的临床资料,观察T细胞/组织细胞丰富的大B细胞淋巴瘤的病理形态学特点及免疫表型。 结果患者男性7例,女性5例,年龄46~78岁;光镜下均可见淋巴结正常结构破坏,在小淋巴细胞和数量不等的组织细胞的背景下,散在单个大细胞;免疫组化显示大细胞CD20阳性,小淋巴细胞CD3阳性。 结论T细胞/组织细胞丰富的大B细胞淋巴瘤是一种相对少见的淋巴瘤,以在小T淋巴细胞和数量不等的组织细胞的背景下散在一定数量的不典型大B细胞为特征;临床及组织学表现均易与其他类型的淋巴瘤相混淆,掌握其共同的特征并结合免疫组化方能正确诊断。     

In situ polymerase chain reaction-based localization studies support role of human herpesvirus-8 as the cause of two AIDS-related neoplasms: Kaposi's sarcoma and body cavity lymphoma.

Several lines of investigation point to a new herpesvirus, human herpesvirus-8 (HHV-8), as the cause of two different neoplasms seen in AIDS patients-Kaposi's sarcoma (KS) and body cavity B cell lymphoma. If this virus is the etiological agent, rather than another opportunistic infectious agent, it should be present in the earliest detectable clinical lesions on a temporal basis, and localize to specific target cells in a spatial pattern consistent with tumorigenic pathways. In this study, we take advantage of the clinical accessibility to biopsy early (patch stage) skin lesions of KS to address the temporal issue, combined with in situ PCR and dual immunostaining using a marker identifying malignant cells, to address the spatial localization issue. 21 different tissue samples were subjected to PCR analysis and in situ PCR with and without simultaneous immunostaining. In normal skin from healthy individuals, no HHV-8 DNA was detected by PCR or in situ PCR. However, in all PCR-positive tissues, distinct and specific in situ PCR staining was observed. In four different patch stage KS lesions, in situ PCR staining localized to nuclei of endothelial cells and perivascular spindle-shaped tumor cells. Later stage KS lesions (plaques and nodules) revealed additional positive cells, including epidermal keratinocytes (four of five), and eccrine epithelia (two of four). These patterns were nonrestricted to skin, as pulmonary KS also revealed HHV-8-specific infection of endothelial cells and KS tumor cells, as well as epithelioid pneumocytes (two of two). In body cavity B cell lymphoma by dual staining, HHV-8 was present in malignant tumor cells (EMA immunostained positive) and not in reactive lymphocytes. These results reveal an early temporal onset and nonrandom tissue and cellular distribution pattern for HHV-8 infection that is consistent with a causal link between this DNA virus and two AIDS-related neoplasms.     

伴皮肤侵犯的鼻NK/T淋巴瘤临床病理分析及免疫组化研究

目的　探讨伴皮肤侵犯的鼻NK T淋巴瘤病理形态学、免疫表型特征。方法　对 3例伴皮肤侵犯的鼻NK T淋巴瘤进行组织形态学观察 ,并行免疫组化检测。结果　 3例患者在确诊鼻NK T细胞淋巴瘤后数月内继发皮肤病变 ,并多在 1年内死亡。皮肤及鼻腔病变组织形态学有共同特征 ,免疫表型均符合结外鼻型NK T淋巴瘤特点 ,LCA、CD2、CD5 6和TIA 1(+) ,CD3胞质阳性 ,胞膜阴性。 3例中有 2例瘤细胞表达EBV ,瘤细胞增殖指数均 >85 %。结论　鼻NK T淋巴瘤是一种高度恶性肿瘤 ,易发生皮肤侵犯 ,预后差 ,其诊断及鉴别诊断有赖于特征性组织形态学及免疫表型特点     

肝血管内大B细胞性淋巴瘤2例临床病理观察

目的探讨肝血管内大B细胞性淋巴瘤的临床病理特征、诊断与鉴别诊断、治疗及预后。方法回顾性分析2例肝血管内大B细胞性淋巴瘤患者的临床资料、组织病理学形态和免疫组化结果。结果光镜下肝窦内和小血管内可见较多具有明显异型性的淋巴样细胞浸润,汇管区可见慢性炎细胞浸润,亦可见少许异型淋巴细胞样细胞,未见明确纤维化。免疫组化示CD20、PAX5弥漫(+),CD3散在少许(+),Ki-67阳性率为70%,AE1/AE3、CD117和CD56均(-);其中例1 CD5弥漫(+)。结论血管内大B细胞性淋巴瘤是一种具有高度侵袭性的结外弥漫性大B细胞性淋巴瘤的亚型,由于该病临床表现多样及不典型性,造成了部分患者的诊断困难,因此,掌握临床病理及免疫组化特征对该病的诊断和鉴别诊断具有重要意义。     

Disseminated intravascular large B cell lymphoma with slowly decreasing high-density lipoprotein cholesterol

Intravascular large B cell lymphoma (IVLBCL) is a rare type of extranodal large B cell lymphoma in the lumina of small vessels. Low high-density lipoprotein cholesterol (HDL-C) is associated with sepsis, malignancy, and death. Recent evidence suggests an inverse relationship between HDL-C and non-Hodgkin lymphoma. We report the case of a 71-year-old female who presented with decreasing HDL-C for years prior to diagnosis of IVLBCL. The patient developed nonspecific symptoms, including dizziness, gait instability, fatigue, tinnitus, and weight loss. Although malignancy was high on the differential, no diagnosis was made antemortem. The diagnosis of disseminated intravascular large B cell lymphoma was made postmortem in multiple organ systems. The presentation of IVLBCL is nonspecific and misleading. To our knowledge this is the second known case report of low HDL-C preceding diagnosis of IVLBCL, but the first case documenting low HDL-C years prior to diagnosis.     

脾上皮样血管内皮细胞瘤3例临床病理分析

目的探讨脾血管内皮细胞瘤的临床病理学特征、诊断及鉴别诊断要点。方法报道3例罕见的脾血管内皮细胞瘤,结合文献对其临床表现、组织形态、免疫组化及治疗预后进行分析。结果男性1例,女性2例,年龄27～51岁,平均年龄35岁。患者因影像学检查示脾占位就诊。大体观察均为局限单个边界清楚肿物。镜下均可见大量血管内皮泡沫样细胞、血管增生及扩张。免疫组化:CD31和CD34(+),CD68局部(+),SMA(■),CK(-)。结论脾上皮样血管内皮细胞瘤是一种罕见的肿瘤,确诊需依靠病理检查及免疫组化,具有细胞内血管腔的上皮样瘤细胞并呈血管内皮标记物阳性是该病病理诊断的两个重要特征。需同脾其他肿瘤鉴别。     

Hemophagocytic syndrome and neurological involvement in a case of intravascular large B-cell lymphoma

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin’s lymphoma, and is divided into Western and Asian variants. The latter is rarely found to have neurological system involvement. In China, there have only been a few diagnosed cases of IVLBCL. Here, we present a Chinese case of Asian-variant IVLBCL with neurological symptoms. A 32-year-old Chinese man presented with bilateral lower limb numbness and persistent fever. He also complained of difficulties in urination and defecation. In addition, splenomegaly and pancytopenia were observed. We identified 3% dysplastic lymphocytes in his peripheral blood film, and his bone marrow biopsy led to a diagnosis of Asian-variant IVLBCL. Lumbar spine magnetic resonance imaging, which revealed an edematous spinal cord, further confirmed neurological involvement. The patient refused treatment from the time of diagnosis, and died 2 months after being discharged. IVLBCL is a highly aggressive but nonspecific clinical manifestation that is difficult to diagnose; therefore, a greater understanding of the disease is needed. The current first-line therapy involves R-CHOP combination therapy (cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab); however, the overall prognosis of IVLBCL remains poor.     

Distribution of vascular permeability factor (vascular endothelial growth factor) in tumors: concentration in tumor blood vessels

Vascular permeability factor (VPF) is a highly conserved 34-42-kD protein secreted by many tumor cells. Among the most potent vascular permeability-enhancing factors known, VPF is also a selective vascular endothelial cell mitogen, and therefore has been called vascular endothelial cell growth factor (VEGF). Our goal was to define the cellular sites of VPF (VEGF) synthesis and accumulation in tumors in vivo. Immunohistochemical studies were performed on solid and ascites guinea pig line 1 and line 10 bile duct carcinomas using antibodies directed against peptides synthesized to represent the NH2-terminal and internal sequences of VPF. These antibodies stained tumor cells and, uniformly and most intensely, the endothelium of immediately adjacent blood vessels, both preexisting and those newly induced by tumor angiogenesis. A similar pattern of VPF staining was observed in autochthonous human lymphoma. In situ hybridization demonstrated VPF mRNA in nearly all line 10 tumor cells but not in tumor blood vessels, indicating that immunohistochemical labeling of tumor vessels with antibodies to VPF peptides reflects uptake of VPF, not endogenous synthesis. VPF protein staining was evident in adjacent preexisting venules and small veins as early as 5 h after tumor transplant and plateaued at maximally intense levels in newly induced tumor vessels by approximately 5 d. VPF-stained vessels were also hyperpermeable to macromolecules as judged by their capacity to accumulate circulating colloidal carbon. In contrast, vessels more than approximately 0.5 mm distant from tumors were not hyperpermeable and did not exhibit immunohistochemical staining for VPF. Vessel staining disappeared within 24-48 h of tumor rejection. These studies indicate that VPF is synthesized by tumor cells in vivo and accumulates in nearby blood vessels, its target of action. Because leaky tumor vessels initiate a cascade of events, which include plasma extravasation and which lead ultimately to angiogenesis and tumor stroma formation, VPF may have a pivotal role in promoting tumor growth. Also, VPF immunostaining provides a new marker for tumor blood vessels that may be exploitable for tumor imaging or therapy.     

上皮样血管内皮瘤临床病理学分析

目的：探讨上皮样血管内皮瘤（EH）的病理形态及免疫组化特点。方法：对5例不同部位的EH进行光镜观察和免疫组化标记,并结合文献进行讨论。结果：5例中男性2例,女性3例,年龄37－75岁,中位年龄54岁,肿瘤发生部位分别是腹壁,肺,阴茎,鼻前庭,骨,光镜下见肿瘤细胞形态多样,排列成巢状,条索状甚至腺样结构。瘤细胞短梭形,胞浆内空泡,此特征为单细胞原始管腔结构,肿瘤细胞表达血管内皮细胞标记如CD34,Ⅷ因子,UEA等,部分病例同时表达CK和／或Vim。结论：EH的病理形态具有一定的特征性,诊断时需要和转移性癌,上皮样血管肉瘤鉴别,同时在概念上需要与上皮样血管瘤区别,本病生物学行为与组织学形态有不一致性,治疗按低度恶性处理。     

伴有明显淋巴滤泡的非特指外周T细胞淋巴瘤

目的　探讨伴有明显淋巴滤泡增生的非特指外周T细胞淋巴瘤的形态学特点和免疫表型。方法　对 3例特殊形态的外周T细胞淋巴瘤进行形态学观察 ,免疫组化EnVision法检测CD3、CD4 5RO、CD4 3、CD2 0、CD79a、cyclinD1、bcl 2、CD4、CD8、S 10 0等抗体 ,并用PCR方法进行T细胞受体(TCR)基因重排检测。结果　 3例患者均以全身浅表淋巴结肿大为主要表现 ,初发部位为颈部和颌下 ,发病过程中出现低热及全身皮疹 ,并有肝、脾肿大。形态学的显著特点为淋巴滤泡增生 ,套区消失 ,边缘区有中等大小、胞浆透亮的瘤细胞浸润生长。免疫表型标志为T细胞淋巴瘤。基因检测有TCRγ基因阳性条带。结论　部分非特指外周T细胞淋巴瘤可伴有明显淋巴滤泡增生 ,应注意与淋巴结反应性增生、滤泡性淋巴瘤、边缘区淋巴瘤及套区淋巴瘤等鉴别